Biochemistry Flashcards by Jehan Salapuddin

UV associated with skin malignancies

UV B (Bad UV light)

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helps predict the direction of equilibrium in change of concentration, pressure, volume, or temperature

le chatelier’s principle

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gains electrons, loses oxygen, stores energy

reduction
GEROA
gains electrons, reduced = oxidizing agent

LEORA
loses electerons, oxidized = reducing agent

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protein Donors

aciDs

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MC organic molecule in body, act as buffers

amino acids

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all AA are chiral except for

glycine (achiral- has 2 H attached)

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non-polar/hydrophobic AA

PT rypGA VLIMP

Phe
Tryptophan

Glycine
Alanine

Valine
Leucine
Isoleucine
Methionine
Proline

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AA found on surface of proteins, hydrogen bond formation

polar AA (hydrophilic, uncharged)

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basic, charged amino acids

HAL
histidine
arginine
lysine

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major inhibitory neurotransmitter of spinal cord

glycine

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tetanospasmin prevents release of __ from renshaw cells which leads to spastic paralysis

glycine

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carrier of ammonia from skeletal muscle to liver

alanine

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branched chain AA

VLI
valine
leucine
isoleucine

*metabolites accumulate in MSUD

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accumultes in PKU, precursor of tyrosine

phenylanine
“pare, true love does not exist”
phe
tyrosine
l-dopa
dopamine
norepi
epi

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precursor of melanine

tyrosine

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precursor for melatonin

tryptophan
“TRYP Mo Sya Noh?”
melatonin
serotonin
niacin

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precursor for homocysteine and cysteine

methionine

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interrupts a-helices in globular proteins

Patay Gutom
proline
glycine

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N-linked glycosylation of proteins

asparagiNe

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major carrier of ammonia from peripheral tissues to liver

glutamine

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keratin has lots of this AA which causes kinking of hair

cysteine bonds

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precursor for GABA and glutathione

glutamate

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major inhibitory neurotransmitter of the brain

GABA

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major excitatory neurotransmitter of CNS

glutamate

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basic AA precursor of nitric oxide

arginine others: crea urea citrulline

precursor of carnitine

Lysine (fit n' right drink has L-carnitine)

semi-essential AA required in adolescence for bone growth

arginine

essential AA

PVT TIM HALL always ARGues, never TYRone PROtacio phenylalanine valine tryptophan threonine isoleucine methionine histidine arginine lycine leucine

most abundant and functionally diverse molecule in humans

proteins

shuttle hydrophobic molecules in the blood

proteins

this is disrupted by hydrolysis but not denaturation

peptide bonds in primary structure

targeting signals and allow N-try to a specific organelle

N-terminus

retention signals and Contains inside organelle

C-terminus

contains alpha-helix and b-pleated sheets

secondary structure of protein - stabilized by hydrogen bonds

example of this structure is hgb which has 2 alpha and 2 beta chains

quarternary structure

prevents protein aggregration, proper folding of proteins

chaperone proteins

unfolding and disorganizing proteins

denaturation

accumulation of this leads to spongiform neurodegeneration of brain leading to various conditions like Creudtzfelt-Jakob, kuru..

PrPsc (prpc normal)

MC and most important degenerative disease of brain

alzheimer's disease

keratin collagen fibers elastin fibers

fibrous proteins - spherical, structural proteins

1 oxygen is bound, oxygen storage in heart and skeletal muscle, saturation

myoglobin

oxygen binds to hgb in 2 conditions

hgb in R state (relaxed) iron in ferrous state (Fe2+), not ferric

produces a strong red fluorescence under UV light, cyclic compound formed by 4 aromatic pyrrole rings

porphyrin

in o2-hgb dissociation curve shift to the right, what happens to o2 affinity?

decrease to let go hgb to tissues "CABET, do the RIGHT thing, LET GO" CO2 acidosis bisphosphonate exercise temperature *all high (low pH)

prolonged high altitude, what happens to 02affinity?

decreases

required in heme formation

glycine

blood cell formation

Young Liver Synthesized Blood Yolk sac - embryo Liver - fetal Spleen - fetal Bone marrow - adult

determines glycemic control of DM over the past 3 months

HbA1C

myoglobin deposits in kidneys cauding tea-colored urine in a patient post-hazing

myoglobinuria (rhabdomyolysis - damaged muscle fibers release myoglobin)

1st biomarker that rises in blood after myocardial necrosis

myoglobin

therapy for carboxyhemoglobin

100% o2 to displace CO

ferric state has a higher affinity for __ causing chocolate cyanosis in methemoglobin

cyanide

this causes irreversible binding to heme leading to greenish discoloration

sulfhemoglobin

inherited defect in RBC leading to loss of biconcavity

hereditary spherocytosis -mutation in ankyrin>spectrin

point mutation from glutamate to valine

sickle cell disease

given in sickle cell disease to increase HbF to prevent sickling of RBCs

hydroxyurea

in Hgb C disease, what AA are involved in mutation

glutamate to lyCine

chromosome affected in a-thalassemia

chromosome 16 (4 alleles) chromosome 11 (2 alleles) - b-thalassemia

hgb H disease

a-thalassemia 3 missing alleles (draw an H, 3 lines yan)

most abundant protein in body

collagen

rich in glycine (G), proline (X), hydroxyproline/hydroxylycine (Y)

collagen

synthesis of preprocollagen is in

rough ER

required in hydroxylation of collagen

vitamin c ferrous iron O2 a-ketoglutarate

menke's disease

copper deficiency - cross-linking problem in collagen synthesis

MC type of collagen deficiency

type I - bone, tendone (osteogenesis imperfecta)

collagen deficiency type of epidermolysis bullosa

type VII -anchorig fibrils (mukhang hook like 7)

MC type of Ehlers-Danlos syndrome, type III defect, early osteoarthritis

hypermobility type

type of EDS with intracranial aneurysms, most severe

Vascular -type III

blue scleare, triple helix formation collagen problem

brittle bone disease (OI)

affected collagen type in Alport and Goodpasture syndromes

type IV Al4t Goodpast4t

hemoptysis hematuria

goodpasture (pulmorenal) syndrome

corkscrew hair, poor wound healing, gum bleed

scurvy

kinky/steely hair

menke's disease - copper def

major AA in elastin

proline and lysine

mutation in fibrillin gene, chromosome 15

marfan syndrome

lens upward and outward long extremities aortic dissection

marfan syndrome ectopia lentis homocystinuria - lens downward and inward

inhibits proteolytic enzymes from hydrolyzing and destroying proteins

a1 antitrypsin

fragment of antibodies that determine the isotype of Ig, heavy chains only

FC fragment

main composition of Bence-Jones proteins in urine of multiple myeloma

kappa and lambda in light chains of immunoglobulin - kappa most abundant

cleaves 3 fragments in immunoglobulin

pa-pa-in pepsin 2 fragments

antigen part where antibody binds

epitope I'm an epitope cos im antijjen paratope - antibody part that binds to epitope

most abundant organic molecule in nature

carbohydrates

major energy source

glucose

storage form of energy

glycogen

polysaccharides are linked together by

glycosidic bonds

oxidation of glucose yields

glucoronidase

storage polysaccharide of plants

starch

polysaccharide of fructose that determines GFR

inulin

found in cell walls of fungi, eoskeleton of crustacheans

chitin

isomers with different configuration in 1 carbon atom except carbonyl carbon

epimers

anomers have different configuration in carbonyl atom, without energy use

mutarotation

only glucose transporter that is insulin-dependent

GLUT4 the muscle and adipose guy

glucose transporter in intestinal LUMEN

GLUT5 - lumen 5 letters ahaha

glucose transport from a higher concentration to a lower concentration

facilitated diffusion

carboxylic acids with long aliphatic chain

fatty acids

long chain fatty acids are transported in __ to lymphaticss

chylomicrons

source of energy for mucosa of colon (colonocytes)

short chain fatty acids

geometric isomerism that is same side ang H-atoms

Cis-form sizturrr trans-form pag opposite

parent compound of arachidonic acid

linoleic acid (essential FA)

parent compound of eicosanoids: PG, thromboxane, prostacyclin, leukotrienes

arachidonic acid

docosahexaenoic acid (DHA) is found in

omega 3 FA this is necessary for fetal brain development and retina

main storage form of lipids

triacylglycerol/triglyceride/neutral fat

efficient transport mechanism for lipids to and from tissues

lipoproteins

largest of lipoproteins, has the lowest density

chylomicrons

highest cholesterol in lipoproteins

LDL source is VLDL

needed to activate Vitamin D

UVB

Nitrogenous bases of PYRimidine

CUT the PYR! Cytosine Uracil - RNA Thymine - TNA

nucleoside linkage between base and sugar

N-glycosidic bond

nucleotide linkage between sugar and phosphate

ester bond

most abundant free nucleotide

ATP

joined by 3'-5' PDE bond

nucleic acids -magbonding tayo 3 to 5! in writing the base, 5' to 3' direction

stronger nucleic acid bases with 3 hydrogen bonds

G=C pag may GC, strong3r

what rule states that # of purines are always equal to # of pyrimidines

Chargaff's rule

MC form of DNA type

DNA B

left-handed DNA type

type Z

most abundant chromatin protein

histones

DNA organization with beads on a string appearance (tasbeeh)

10 nm chromatin fibril

chromosomes are condensation of DNA during what mitosis phase

prophase

most heterogenous of RNA

mRNA - only protein coding

most abundant RNA

rRNA

1st step in treatment of malnutrition

nutritional status assessment

2.5 yo with wasting, what parameter will be measured

weight for height <2 for length

PEM with fatty liver

kwashiorkor Marasmus for Muscle wasting

what is preferred to measure in central/abdominal obesity

waist circumference

assess nutrient intake of healthy people, not the sick

DRI dietary reference intake

sufficient to meet requirements of 95% of people

RDA recommended dietaey allowance Adequate Intake if no RDA

calories required in a sedentary person

30 kcal/kg/day 35 for moderate active 40 for very active

recommended fat content of food

9kcal/g protein and carbs 4 alcohol 7

Percentage of resting metabolic rate

60-75%

acceptable carbohydrates in food eaten

60-75% fats 20-35% protein 10-35% simple sugars and saturated fats <10%

reduces postprandial blood glucose and produces sensation of fullness

dietary fiber

vitamins prone to toxicity

ADEK (pag adek, mas toxic) fat-soluble vitamins

B12 lasts for ___ in storage sa liver

3-4 years B9 3-4 months

storage site of vit A

stellate cells of Ito in liver (A-to)

decrease in this vitamin increases the risk for measles infection

vitamin A

MC vitamin deficiency worlwide

Vit A deficiency

earliest sign of Vit A deficiency

loss of green light sensitivity night blindness - earliest symptom

test to measure vitamin D

1,25 dihydroxycholecalciferol - most abundant form

most potent form of Vitamin E

a-tocopherol

most potent antioxidant

vitamin E others: A C E Selenium zinc

deficiency of this vitamin leads to loss of position and vibration sense, acanthocytosis of RBCs

vitamin E

toxicity of this vitamin leads to kernicterus

vitamin K

corkscrew hair bleeding diathesis weak immunity

vitamin c deficiency/scurvy

vitamin involve in decarboxylation reaction

thiamin/B1 B7/biotin - carboxylation

synthesis of tryptophan requires 2 vitamins

B2 and B6

confusion ophthalmoplegia ataxia

wernicke encephalopathy (dry beriberi) - reversible korsakoff syndrome - irreversible confabulation memory loss personality changes *common in chronic alcoholism and malnourished

gout in 1st MTP joint

podagra (B3 toxicity)

gopalan's burning feet syndrome

PAAntothenic acid (pentathenic/B5)

sensory neuropathy

B6 toxicity

excessive ingestion of raw egg whites causes B7 deficiency because of __

avidin avidly binds to biotin

high homocysteine high methylmalonic acid low folic acid

B12 deficiency B9 - normal mma

folic acid supplementation for mother with no history of previous neural tube defect baby

0.4mg/400 ug

sulfur is a type of what mineral

macromineral - >100mg/day

iron is a type of what mineral

micromineral (trace elements)

ultra-trace elements

IMS iodine molybdenum selenium

iron for storage and transport state

ferric iron (Fe3+)

major site of iron absorption

proximal duodenum

facilitates ferrous iron absoprtion in enterocytes

DMT-1

chief regulator of systemic iron

hepcidin

do not administer iron in ongoing bacterial infection. T or F

TRUE they love iron and would reproduce faster

index of iron overload

hemosiderin

cofactors in CytoplaZmic superoxide dismutase

copper and zinc

kinky/steely hair

menke's disease - cu deficiency

kayser-fleischer rings, neuropsychiatric symptoms

wilson's disease - excess copper

vesicles with pustular crusting and erythematous face, limbs, perineum, diarrhe, alopecia

zinc deficiency - acrodermatitis enterohepathica

cofactor in Mitochondrial SOD

Manganese

oooxidizes enzymes

Mooolybdenum

RNA with catalytic activity

ribozymes

active enzyme with protein and non protein

holoenzyme apoenzyme - protein only

nonprotein component of enzyme

cofactor/coenzyme

rate of reaction dependent to substrate concentration

1st order kinetics

rate of reaction independent to substrates

zero-order kinetics

substrate concentration at which Vi is half of Vmax

Km - Michaelis constant

increased Km Vmax unchanged competes at binding site what type of inhibitor?

competitive inhibitor

Km unchanged decreased Vmax inhibits catalyst enzyme what type of inhibitor?

noncompetitive inhibitor

regulated by effectors and catalyzed the rate-limiting step

allosteric enzymes

slowest step in metabolic pathway

rate-limiting enzyme

forms that can be absorbed by enterocytes in protein absorption

AA and peptides

the first enzyme that digest starch in oral cavity

salivary amylase/ptyalin

major digestion of carbohydrates take place in small intestine via the enzyme ___

pancreatic amylase

cotransporter at luminal side of enterocyte for carbohydrates absorption

GLUT5 - lumen 5 letters y'all

cotransporter at the basement membrane of enterocytes which releases glucose to the bloodstream

GLUT2 - BM 2 words y'all

capable of hydrolyzing TAG to free fatty acids and 2 monoacylglycerides

pancreatic lipase

bile acids emulsifies products of lipolysis by forming __

micelles

glycerol is directly absorbed in the bloodstream via what transporter

aquaporin transporters then goes to hepatic portal vein

primary immune system organs

thymus bone marrow

secondary immune organ responsible for nonspecific filtration of macrophages

Lymph nodes

what part of lymph node is B cell localized

follicle same with spleen

responsible for removal of encapsulated bacteria

spleen

encapsulated bacteria with no available vaccine due to its high antigenic variation

N.gonorrheae

only lymphocyte part of innate immunity

NK cells

most predominant cell in the 1st 6-24 hrs of infection

neutrophils

becomes macrophages, predominant after first 6-24 hrs of infection

monocytes

major cells against helminthic infections and clears peripheral immune complexes

eosinophils

cells that increase in systemic allergic reactions and in chronic myelogenous leukemia

basophils

causes of eosinophilia

PACCMAN parasites asthma churg-strauss syndrome chronic adrenal insufficiency myeloproliferative disease allergy neoplasia - Hodgkin's

increases in local tissue allergic reaction and in type I hypersensitivity reax

mast cells

major APC that links innate and adaptive immunity

presents degraded viral/bacterial products to lymphocytes to initiate adaptive immunity

destroy antibody-dependent cell-mediated cytotoxicity (ADCC) thru perforin and granzymes

NK cells

destroys endogenous antigens such as viruses

cyt8toxic T cells - CD8 MHC I (I8) exogenous bacteria - hellper T cell (MHC II/CD4)

suppresses T cells during the termination of inflammation

regulatory T cells

produces antibodies/immunoglobulins

plasma cells - clock-face appearance

ID holders of antigens, present fragments to T cells`

MHC T cells - security guard APC - student

Ig most abundant in serum

IgG G>A>M>D>E

Ig capable of complement activation

IgM and IgG

Ig checked in congenital infections and also the first to encounter with any pathogen

IgM

makes bacteria "yummy" for phagocytosis

opsonization

complement pathways all converge in __

C3 convertase

terminal complement proteins for MAC

C5b, C6, C7, C8, C9

complement deficiency that is high risk for recurrent pyogenic URTI and LRTI, and SLE

Early complement deficiency (C1-C4)

complement deficiency causing recurrent neisseria bacterial infection due to defective MAC

late/terminal complement deficiency (C5-C9)

complement deficiency most common worldwide

C2 deficiency - remember C2 drink known worldwide haha

complement deficiency causing herediatry angioedema due to unregulated kalikrein leading to increased bradykinin

C1 esterase inhibitor deficiency

Final common pathway by which electrons from different fuels of body flow to oxygen

Electron transport chain

Complexes of ETC are found in

Inner mitochondrial membrane

Final electron acceptor in ETC

Oxygen

All components of ETC are fixed in inner mitochondrial membrane except for ___ and ____

Coenzyme Q and cytochrome C

Ozidative phosphorylation happens in

Complex V/ATP synthase

If NADH is used in ETC, how many ATPs are produced?

2.5

If FADH2 is used in ETC, how many ATPs are produced?

1.5 ATP

ATP production in tissue hypoxia

Substrate level phosphorylation - anaerobic glycolysis

What complex is inhibited by hydrogen sulfide?

IV others: Cyanide Carbon monoxide Amytal - I Malonate - II DIMERCAPROL - III

decreases ATP production by increasing permeability of inner mitochondrial membrane

Uncouplers

Effects are: Increased O2 consumption Decreased NADH/NAD and FADH/FAD ratios Decreased ATP

Uncouplers If low, High, Low - ETC inhibitors

Heat production of neonates

Nonshivering thermogenesis - they cannot shiver, this decreases ATP synthesis

Example of drug that directly inhibits ATP synthase/Complex V

Oligomycin

Inhibits oxidative phosphorylation by inhibiting transporter of ADP to ATP

Atractyloside

Reactive oxygen species are unstable product produced by ETC which in turn generates 3 toxic products

Superoxide Hydrogen peroxide Hudroxyl-radical

Happens in post-MI patient where the coronary artery blocked was thrombolyzed and causes disruption of cell membranes thru peroxidation

Reperfusion injury

Oxidative phosphorylation for it to occur requires these 2

Oxygen and mitochondria

Substrate phosphorylation occurs in both

Cytosol and mitochondria

Mitochondrial DNA is inherited from

Moms solely

krebs cycle is a type of what metabolism

amphibolic - it doesn'st stop

2nd messenger of glucagon

cAMP

2nd messenger of insulin-growth factor

tyrosine kinase

terminology when a phosphate is added to enzymes

kinase

in these sites, insulin is needed for glucose to enter the cell

adipose and muscle tissues

transporter for adipose and skeletal muscle

GLUT4 (yung mga needing insulin 4 glucose to enter)

major pathway for glucose metabolism

glycolysis

end-products of glycolysis

pyruvate or lactate

rate-limiting enzyme of glycolysis pathway

PFK-1

increased in absorptive state

insulin and glucose (well-fed)

how many ATPs produced and NADH in glycolysis

4 ATPs 2 NADH

glucokinase is found in __ and __; glucose stored for future use after a heavy meal

liver and pancreas

low km, low Vmax, high affinity, for immediate use of glucose only

hexokinase (skyflakes)

most important activator of PFK-1

fructose-2,6-bisphosphate procused by PFK-2

formation of pyruvate is inhibited by __ in fasting state

glucagon

end-product of anaerobic glycolysis

lactate aerobic - pyruvate

shuttle that carries NADH to enter inner mitochondrial membrane for ETC in brain

mas safe! malate aspartate shuttle _ NADH pa din lalabas glycerophosphate shuttle - becomes FADH pagkalabas (holdup-er)

net ATP yield of anaerobic glycolysis

enzyme that enters acetyl CoA to Kreb's cycle to produce ATPs

pyruvate dehydrogenase (pinakamaarte)

coenzymes/cofactors of pyruvate dehydrogenase

4 B vitamins + lipoic acid Thiamin FAD NAD Coenzyme A

the coenzymes of pyruvate dehydrogenase are also required by an enzyme in Kreb's cycle

a-ketoglutarate dehydrogenase

MC enzyme defect in glycolysis

pyruvate kinase deficiency

MCC of congenital lactic acidosis

pyruvate dehydrogenase deficiency

enzyme deficiency that causes low exercise capacity

muscle PFK deficiency

mutations that decreases glucokinase, asymptomatic except during pregnancy

maturity onset diabetes if the young type 2

products of citric acid cycle

2 CO2, 3 NADH, 1 GTP, 1 FADH

rate-limiting enzyme of tricarboxylic acid cycle

isocitrate dehydrogenase

ATP yield of TCA

10 ATPs

enzyme that produces NADH and FADH

dehydrogenase

In kreb's cycle, GTP is produced by

Su-Su Succinyl CoA & Succinate

KREB'S CYCLE PANGMALAKASAN

Acetyl Cidney Is Kind So She Forgives More Often Acetyl CoA Citrate Isocitrate a-Ketoglutarate Succinyl CoA Succinate Fumarate Malate Oxaloacetate

enzyme in krebs which can be inhibited by fluoroacetate

aconitase - acts on citrate to isocitrate

inhibitor of a-ketoglurate dehydrogenase in TCA

arsenite and ammonia - same with pyruvate dehydrogenase

complete oxidation of glucose total ATP yields

30 or 32

replenish intermediates that have been used for biosynthesis of glucose, fatty acids, and amino acids

anaplerotic reactions

most important enzyme in anaplerotic reaction that maintains adequate concentration of oxaloacetate for krebs

pyruvate carboylase

synthesize new glucose from non-carbohydrate precursors

glucoNEOgenesis

occur both in mitochondria and cytosol

HUG heme synthesis urea cycle gluconeogenesis

most important substrate of gluconeogenesis

glucogenic amino acids

rate limiting enzyme of gluconeogenesis

fructose 1,6-bisphosphatase - reverses PFK-1 of glycolysis

pyruvate carboxylase requires __ and ATP

biotin

final step of gluconeogenesis shared with glycogenolysis

conversion of G6P to glucose

gluconeogenesis depends on ___ to burn fat

b-oxidation

in skeletal muscle, lactate is transported to liver where it is converted back to glucose thru gluconeogenesis

cori cycle "si liver martyr, bigay nang bigay lang!"

when blood glucose reaches this level, glucosuria occurs

>10 mmol/L (renal threshold)

90% is found in muscles, 10% in liver

glycogen

glycogen storage lasts for

18-24 hrs

enzymes of glycogenesis

glycogen synthase branching enzyme

mobilizes glycogen back to glucose

glycogenolysis

end product of glycogenolysis

glucose in liver, glucose-6-phosphate in muscle

important enzymes of glycogenolysis

glycogen phosphorylase (parang si pacman kain nang kain) debranching enzyme

acid maltase deficiency leading to accumulation of glycogen in lysosomes

pompe disease

well-fed state, is there phosphorylation?

Well Fed Walang Fosphorylation insulin active glycogenesis Fasting Fosphorylation glucagon glycogenolysis glycogen phosphorylase

glycogen storage disease: decreased G6P

type I Von Gierke disease hepatomegaly hypoglycemia

debranching enzyme deficiency

cori disease type IIIa

glycogen phosphorylase (pcman) deficiency, poor exercise tolerance, high muscle glycogen

type V - McArdle syndrome

GALT deficiency causing cataracts, and symptoms after breastfeeding

classic galactosemia - absolute CI to BF

fructose phosphorylation enzyme

fructokinase/hexokinase

enzyme in formation of DHAP and glyceraldehyde

aldolase B

absorbed faster than glucose

fructose

profound hypoglycemia and vomiting after consumption of fruit juice

aldolase B deficiency (hereditary fructose intolerance) - sx appear after weaning of infant

enzyme that converts glucose to sorbitol which is found in the lens, retina, schwann cells, and kidneys

aldose reductase

in diabetics, sorbitol accumulates in retina, lens, kidneys, and schwann cells due to absence of what enzyme

sorbitol dehydrogenase - converts sorbitol to fructose

main pathway for production of glucoronic acid and iduronic acid

uronic acid pathway

ascorbic acid is needed in the diet as humans do not have the enzyme necessary for its synthesis

L-gulonolactone oxidase

required in detoxification reactions of bilirubin, steroids..

glucoronic acid

high xellulose in urine

essential pentosuria - xellulose reductase deficiency

aka hexose monophosphate shunt, produces NADPH

PPP pentose phosphate pathway

rate limiting enzyme of PPP

glucose-6-p dehydrogenase

what phase of HMP wherein it produces 3 NADPH and ribulose 5-P

1st phase - oxidative/irreversible

essential for glutathione reductions inside RBCs

NADPH

cofactor of glutathione peroxidase

selenium

AA of glutathione

GCG glycine cysteine glutamate

removes H202 via glutathione peroxidase

reduced glutathione

MC disease producing enzyme deficiency, decreased NADPH in RBCs

G6PD deficiency

MC precipitating factor of G6PD deficiency

infection

heinz bodies and bite cells

G6PD deficiency

severe, persistent, chronic pyogenic infections by catalase-positive bacteria, decreased NADPH oxidase

chronic granulomatous disease

Where is fatty acids primarily synthesized?

Liverrrrr

Product of lipogenesis

Palmitate from acetyl CoA

Fatty acids are stored as

Triacylglycerol

Enzyme for degrading truacylglycerol in adipose tissue

Hormone sensitive lipase

Fatty acids undergo ___ to yield acetyl CoA, NADH, FADH2

B oxidation

Alternate fuel during prolonged fasting state

Ketone bodies

Occurs when rate of ketogenesis exceeds its use

Ketoacidosis

activation of fatty acids needs what vitamin

B5 pantothenic acid

the cofactor of the rate limiting enzyme in lipogenesis

biotin (acetyl coA carboxylase)

acetyl coA needs needs __ to go out of mitochondria to cytosol

citrate shuttle

steps in elongation to palmitoyl CoA

Code ReD Red Condensation Reduction Dehydration Reduction *repeated 7x until malonyl CoA

lipid synthesis occurs in

smooth ER

stored fatty acids

triacylglycerol

rate-limiting enzyme in b-oxidation

carnitine-palmitoyl transferase (pampapayat) occurs in mitochondria - mainit like sauna kasi pampapayat!

steps in degradation of fatty acyl coA

OHOT Oxidation Hydration Oxidation Thiolysis

ATP yield of palmitate

106 ATPs

FA with odd number of carbons - 3 carbons

propionyl CoA

MC inborn error of FA oxidation

median-chain fatty acyl CoA dehydrogenase (MCAD)

breastmilk is rich in this kind of fatty acids

short and medium chain FA

toxin of Jamaican vomiting sickness inhibiting B-oxidation

hypoglycin - hypoglycemia

RAtinitis Figmentosa, accumulation of phytenic acid

RAFsum disease

absence of peroxisomes, cerebrohepatorenal syndrome

zellweger syndrome

inability to transport very long chain fatty acids

adrenoleukodystrophy

dietary therapy to decrease synthesis of very long chain fatty acids

lorenzo's oil

CFTR mutation in cystic fibrosis

phenylalanine deletion at codon 508

diagnosis for cystic fibrosis

sweat chloride >60 mmol/L

substrates of acetyl CoA used for fuel

acetoacetate b-hydroxybutyrate

rate-limiting step of ketogenesis

HMG CoA synthase - panggawa ng kHetoneS

how many ATPs per acetyl CoA is produced?

liver produces ketone bodies but cannot use it for fuel due to lack of enzyme __

thiophorase (succinyl CoA acetoacetate CoA) - liver as martyr bigay lang nang bigay si liver nagsaing, si Ralph ang kumain haha

in chronic alcoholism, excess NADH shunts ___ in TCA to malate

oxaloacetate - wala na so walang susundo kay acetyl CoA kaya Krebs cannot be completed

MCC of DKA in young patients

poor adherence to insulin therapy

reason of hypokalemia in DKA

no insulin, no inward shift of K to cells

in Na nitroprusside test for DKA, it measures acetone and acetoacetate, not __

b-hydroybutyrate - kaya dapat direct measurement in urine or serum if na nitroprusside is negative

major sterol in humans and synthesized by almost all tissues

cholesterol

keeps lipids soluble in plasma and for transport to and from tissues

lipoproteins

bile salts are attached to these 2 amino acids

taurine and glycine - prefix glyco- or tauro-

rate-limiting enzyme of cHolesteRol synthesis

HMG-CoA Reductase HMG-CoA to mevalonate

competitive inhibitors of HMG-CoA reductase

statins

multiple congenital malformations, upturned nose

smith-lemli-opitz syndrome - deficient 7-dehydrocholesterol reductase which is needed for the final step in cholesterol synthesis

prevents hypoglycemia in stressful situations

cortisol *glucagon - prevents it during fasting

hypotension, salt-wasting form of CAH

21-a-hydroxylase deficiency low-renin hypertension (11 B-1 hydroxylase deficiency)

lipoprotein with lowest density, highest TAG

chylomicrons

lipoprotein with highest protein and density

HDL the good guy

increased HDL

familial hyperalphalipoproteinemia

Disorders in heme synthesis lead to

Porphyrias

Nitrogen intake > excretion like in a growing child

Positive nitrogen balance

If dumikit sa protein, katapusan nya na

Ubiquitin - for protein denaturation na

Excess proteins are not stored. T or F

True, it is thrown away

Protein synthesized per day

300-400g

Majority of these are converted to urea

Ammonia

Coenzyme of aminotransferase

Pyridoxal phosphate

Glutamate is deaminated releasing ammonia by what enzyme

Glutamate dehydrogenase

Glutamine releases ammonia in __ and __

Liver and kidneys in response to a protein-riched intake or metabolic acidosis

AA derived to form urea and regenerate ornithine

Arginine

3 AA that binds with ammonia

Glutamate - ultimate basurero Glutamine Alanine

MCC of hereditary hyperammonemia

Ornithine transcarbomylase deficiency (ammonia, orotic acid)

Hyperornithinemia Huperammonemia Homocitrullinemia

HHH sydrome

Unable to detoxify ammonia to liver due to blood bypassing liver

Acquired hyperammonemia

Glucogenic and ketogenic AA

TRy PHilippine ISlands, TY! Tryptophan Phenylalanine Isoleucine Tyrosine *threonine - other books Ketogenic Leucine Lysine

AA with glutathione byproduct

Cysteine Glutamate Glycine

Precursor of melaTOnin

trypTOphan melanin- tyrosine

Sythesized from methionine and ATP

S-adenosylmethionine (SAM)

Black urine when standing

Alkaptonuria - accumulation of homogentisic acid

Absence of copper-requiring enzyme tyrosinase leading to increased risk for skin cancer

Albinism

Cabbage-like odor

Tyrosinemia type I

Downward displacement of lens

Ectopia lentis in homocystinuria Upward displacement in Marfan's

Inherited defect in PCT of kidneys Cystine Ornithine Lysine Arginine

Cystinuria

Methylmalonaye acidemia that should be avoided

MVIT Methionine Valine Isoleucine Threonine

Blocked degradation if leucine, isoleucine, valine

MSUD - branched chain AA

80% of heme synthesis occurs in

Bone marrow

Inhibited by heavy metal ions and replaces zinc

PBG Porphobilinogen

Increases uptake of iron from intestinal tract

Vitamin C

Lead poisoning inactivates

ALA dehydratase

MCC of porphyria in adults

Porphyria cutanea tarda

MC porphyria in children

Erythropoietic protoporphyria (EPP)

Urine darkens on exposure to light and air

Acute intermittent porphyria

Bilirubin is transported to liver by binding to

Albumin

Enzyme that conjugates bilirubin ti bilirubin diglucoronide

Bilirubin glucuronulytansferase

Most lethal form of hereditary hyperalbuminemia >20-50mg/dL

Crigler-Najjar syndrome type I

Both type I and II Crigler- Najjar syndrome require __

phototherapy

In alcohol metabolism, what inhibits the enzyme aldehyde dehydrogenase

Disulfiram - causes hungovet

Alcohol component that causes hungover

Acetaldehyde

Most active form of vitamin D

1,25 dihydroxyvitamin D

Neural tube closes at

Day 28 gestation

Test for B12 deficiency

Schilling test

Test for folate deficiency

FIGLU formiminoglutamic acid secretion test

All glycosaminogkycans are attached to proteins forming proteiglycans except

Hyaluronic acid

Most abundant GAG

Chondroitin sulfate - found in tendon and cartilage

All GAGs are extracellular except for

Heparin

Accumulation of GAGs in lysosomes due to deficient hydrolases

Mucopolysaccharidoses

Type of MPS with No corneal clouding

MPS II Hunter syndrome they need sharp eyes

Type of MPS with no CNS involvement

Type IV Morquio syndrome

Only significant sphingomyophospholipid

Sphingomyelin

Erlen meter flask femur

Gaucher's disease - sphingolipidoses; crumpled tissue paper like

Foam cells, hepatosplenomegaly with cherry red spot, increase sphingomyelin

Niemann pick disease

Chery red spot, no hepatosplenomegaly

Tay-Sachs disease

Blood type, N-acetyl-galactosamine found on surface

Type A

Parent molecule of purine synthesis

Inosine monophosphate

Rete limiting enzyme of purine synthesis

PRPP glutamylamidotransferase

Rate limitingbenzyme of pyrimidine synthesis

CPS II CPS 1 - urea cycle

Converts ribonucleotides to deoxyribonucleotides

Ribonucleotide reductase

Product of purine degradation

Uric acid

Product of pyrimidine degradation

Orotic aciduria - harmless

Purine salvage enzymes

Hypoxanthine-guanine phosphoribosylytransferase (HGPRTase) and APRTase

Drug with Non-purine inhibitor of xanthine oxidase

Febuxostat

Drug that reversibly inhibits IMP dehydrogenase

Mycophenolate

HGPRTase deficiency leading to gout and self-mutilation

Lesch-Nyhan syndrome

Adenosine deaminase deficiency leading to overwhelming infection

SCID

Glucose-6-phosphate dehydrogenase deficiency

Von gierke disease

Chargaff's rule only applies to

DNA, not in RNA yung purine=purine, pyrimidine=pyrimidine

Most abundant RNA, formation and function of ribosomes

RNA

All RNA viruses are single-stranded except

Reovirus

DNA replication occurs during what phase of mitosis

S phase

__ unwinds the DNA double helix

Helicase

Relieve torsional strain resulting from unwinding of DNA helix

Topoisomerase

Elongates DNA strand, puts primer

DNA polymerase III - tamad

Okazaki fragments are found in what strand

Lagging strand

Fills the gap in DNA strand, removes primers

DNA polymerase I

A=C mismatched strand is prone to

Lynch syndrome - hereditary nonpolyposis colon cancer

Disease in pyrimidine dimers, cause is exposure to UV light

Xeroderma pigmentosum

Inhibits topoisomerase

Etoposide

No primer need and proofreading, prone to error

Transcription (DNA to RNA)

What is switched on when lactose is present and hypoglycemia

Lactose operon

What gene encodes for entry of lactose into cell

Y gene

Lac repressor protein

I gene - prevents transcription

Stop codons

UAG UGA UAA

Start codon

Ang Unang Gumagawa

What degrade defective protein

Proteosomes

Inhibitors of transcription

Rifampicin and dactinomycin

Bind to 30s subunit, inhibits initiation of translation

Streptomycin and aminoglycoside

Bind to 50s subunit, inhibits translocation

Clindamycin and macrolides

Mutation purine to purine, pyrimidine to pyrimidine

Point mutations A to G, C to T

New codon is a stop codon (UAG/UGA/UAA)

Nonsense

Mutations with deletion or addition of bases

Frame shift

Repeated gene in huntington's

CAG Fragile X syndrome: CGG

Biochemistry Flashcards

(450 cards)