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Nutrition And Metabolism > Protein And Amino Acid Metabolism > Flashcards

Protein And Amino Acid Metabolism Flashcards

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1
Q

What happens to excess amino acids in the body

A

Cannot be stored so used for fuel

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2
Q

Kwashiorkor

A

Protein deficiency but adequate calories

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3
Q

Famine edema

A

Inadequate synthesis of plasma proteins like albumin so fluid Escape into tissues (oncotic pressure)

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4
Q

Marasmus

A

Protein calorie deficiency

Starvation

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5
Q

People at risk of protein malnutrition

A

Pregnant and lactating women

individuals with eating disorders

chronic alcoholics

substance abusers

hospital patients with major protein
needs

elderly

clinical chronically ill

individuals patience with genetic disorders in amino acid metabolism

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6
Q

Essential amino acids (PVT TIM HALL)

A

Phenylalanine
Valine
Tryptophan

Threonine
Isoleucine
Methionine

Histidine
Arginine
Leucine
Lysine

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7
Q

Is arginine always an essential amino acids

A

No

essential in children because of active growth but nonessential in adults

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8
Q

What is a glucogenic Amino acid

A

Product can enter gluconeogenesis

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9
Q

What is a ketogenic amino acid

A

Product intermediate of lipids metabolism or Ketone bodies

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10
Q

Positive nitrogen balance

A

More intake than excretion

Growth, pregnancy, tissue repair

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11
Q

Negative N balance

A

N excretion > intake

Starvation
Malnutrition
Illness
Surgery

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12
Q

General rédaction of amino acids

A

Transamination

Oxidative deamination

Decarboxylation

Transdeamination

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13
Q

Transamination

A

Caralyzed by transaminases (aminotransferase)

Transfer of NH2 to produce another AA

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14
Q

Co factor of transaminases

A

Pyridoxal phosphate from vit B6

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15
Q

Main compound in AA metabolism

A

Glutamate
Glutamine
a-KG

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16
Q

Neutral transport and storage form of ammonia

A

Glutamine

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17
Q

Glutamate dehydrogenase rxn

A

a-KG ——-> glutamate by glutamate dehydrogenase

Glutamate ——> glutamine by glutamine synthétase

Glutamine —-> glutamate by glutaminase

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18
Q

Genetic disorder in which glutamate dehydrogenase always activated due to binding site of GTP mutation

A

Hyperinsulinism - hyperammonemia syndrome with hypoglycemia

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19
Q

Where in the body do you find amino acids oxidase

A

The kidney

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20
Q

Decarboxylation of AA

A

Removal of CO2 by glu decarboxylase to form GABA

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21
Q

Transdeamination

A

Coupling of aminotransferase

with glutamate dehydrogenase reaction ( transamination+ deamination )

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22
Q

Tryptophan metabolized to

A 
97%: 
Alanine 
Acetyl Coa
CO2 
Formate

3% :
Melatonin
Serotonin
Nicotinate ( niacin )

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23
Q

Serotonin function

A 
Neurotransmission 
Behavioral processes (!appetite, agression , sleep ,sensory perceptions, depression 
Vasoconstriction
Regulate circadian cycle , intestinal peristalsis
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24
Q

Agonist of serotonin

A

LSD

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25
Antidepressants and their MOA
Paxil Prozac Zoloft Inhibits serotonin reuptake
26
Hartnup disease
Defect in transportation or absorption of amino acids in intestine Leads to essential amino acid deficiency and nicotinamide deficiencies
27
Hartnup disease symptoms
Hereditary pellagra like skin rash Temporary cerebellum ataxia Renal amino aciduria
28
Hartnup disease 3 D’s
Dermatitis Diarrhea Dementia
29
What factors precipitate hartnup disease
``` Sunburn Fever Inadequate nutrition Irregular diet Stress ```
30
Under what form is NH3 excreted
Urea
31
Direct sources of ammonia in liver
Glutamate Glutamine (extrahepwtic tissues ) Alanine (muscles )
32
Organs that contributes the most to waste nitrogen
Muscles
33
Allostérie activator of CPSI
N acetylglutamate
34
Difference between CPSI and CPSII
CPSI only in mitochondria and uses NH3 in urea cycle CPSII only in cytosol and uses glutamine in pyrimidine synthesis
35
Organ where urea cycle takes place
Liver
36
Reactions of urea cycle in mitochondria
Ammonia —-> carbamoyl phosphate By CPSI Carbamoyl phosphate —-> citrulline By ornithine transcarbamoylase and addition of ornithine
37
Urea cycle rxn in cytosol
Citrulline —-> argininosuccinage By argininosuccinate synthase and addition of aspartic acid Argininosuccinate —-> arginine By argininosuccinase and removal of fumarate Arginine ——> ornithine By arginase and removal of urea
38
Fate of fumarate in urea cycle
Glucose source Aspartate source Energy source
39
2 hypothesis of urea malfunctions leading to coma
Depletion of a-KG because too much NH4 allow conversion of a-KG to glutamate by glutamate dehydrogenase. So less a-KG free for TCA cycle -> less oxidative phosphorylation which is important for neurons Toxicity of glutamine due to its accumulation in astrocytes. Leads to osmotic pressure -> swelling -> edema
40
Clinical manifestations of urea cycle malfunctions
``` Vomiting Seizures Somnolence Coma Death ```
41
Most common urea cycle enzyme malfunction
Ornithine gras carbamoylase
42
Symptoms of hyperammonemia type 2 (ornithine transcarbamoylase deficiency)
Mild : episodic hyperammonemia when high protein intake or infection Severe : neonatal hyperammonemic coma, death , mental retardation, cerebral palsy when survive High glutamine level
43
Hyperammonemia type 1 due to
CPSI deficiency
44
Citrullenemia
Hyperammonemia + high citrulline level | Due to argininosuccinate synthase deficiency
45
Management of citrullinemia
Arginine feeding (enhance citrulline excretion ) Benzoate feeding (divert ammonia to hyppurate)
46
Emergency Management of hyperammonemia
IV glucose and lipid (stop protein catabolism ) Hemodialysis Phenylacetate for gln conjugation-> enhance n acetylglutamate synthase
47
Long term Management of hyperammonemia
``` Less dietary protein intake Adequate essential AA Non protein calories Arginine or citrulline if necessary Drugs (phenylbutyrate , benzoate) ```
48
Phenylalanine main product
Tyrosine
49
Tyrosine metabolism end product
Fumarate + acetoacetate
50
Phenylalanine metabolism overview
Phe ——> tyrosine By phe hydroxylase + H4biopterin Tyr——> p hydroxylphenyl pyruvate By aminotransferase hydroxylphenyl pyruvate —-> homogentisate By oxidase Homogentisate —-> maleyl acetoacetate By oxidase Maleyl acetoacetate ——> fumaryl acetoacetate By isomerase Fumaryl acetoacetate——> acetoacetate + fumarate By hydrolase
51
Tyrosinemias
Deficiency in tyr aminotransferase | Accumulation of tyr and metabolites
52
Type II Tyrosinemias (oculocutaneous)
Eye & skin lesions Mental retardation Cornea keratitis Palm hyperkeratosis +ulcers
53
Type I tyrosenemias (hepatorenal )
Liver failure Renal tubular dysfunction Rickets Polyneuropathy Caused by fumarylacetoacetate hydrolase deficiency
54
Type I tyrosinemia management
Diet Nitisone Liver transplant
55
Alcaptonuria
homogentisate oxidase deficiency so accumulation of homogentisate in urine Urine looks intense dark color Deposition of dark pigment in cartilage and connective tissue
56
Albinism
Deficiency of tyrosinase So no skin or hair color Sun sensitivity Photophobia
57
Phenylketonuria
``` Phenylalanine hydroxylase deficiency (classical) Or H4biopterin deficiency (non classical) ```
58
Phenylketonuria symptoms
Mental retardation Seizures Microcéphaly
59
PKU type I management
Low diet in phe Adequate diet with tyrosine Screening of neonate so that therapy can work Long term maintenance of diet
60
Tyrosine precursors of
Catecholamines ( l dopa, dopamine , norepinephrine , epinephrine
61
Treatment of Parkinson’s disease
Dopa because can cross blood brain barrier ( dopamine can’t )
62
If high level of homovanillate , what does it mean
Dopamine metabolism malfunction
63
If high level of vanillykmandelate, what does it mean
Epinephrine and norepinephrine metabolism malfunction
64
Branched chain AA
Leucine Isoleucine Valine
65
Leucine catabolism
Leucine ——> isovaleryl coa ——> acetoacetyl coa
66
Valine and isoleucine catabolism
Aa —-> some buturyl stuff ——-€ propionyl COA —biotin—-> methylmalonyl coa ——> succinyl coa
67
Disorders of BCAA
Propionicacidemia Maple syrup disease Isovaleric acidemia Methylmalonicacidemi
68
Propionic acidemia
Deficient carboxylase or biotin preventing formation of methylmalonyl coa So propionyl coa accumulation
69
Propionicacidemia symptoms
Hypoglycemia Hyperammonemia Less succinyl coa
70
Maple syrup urine disease
Val île leu metabolism blocked because ketoacid dehydrogenase deficient So hypoglycemia and ketoacidosis Mental retardation
71
Isovaleric acidemia
Isovaleryl coa dehydrogenase deficiency Isovaleryl coa accumulation So form toxic metabolites in kidney (isovaleryl glycine, isovalerylcarnitine )
72
Isovaleric acidemia symptoms
Coma Acidosis Ketonuria Sweaty feet odour
73
Isovaleric acidemia management
Diet management Newborn screening Crisis management
74
Methylmalonic acidemia
Defective methylmalonyl mutass or cobalamin | So methylmalonyl coa accumulation which inhibits pyruvate carboxylase
75
Methylmalonyl acidemia symptoms
``` Hyperammonemia Coma Nephropathy CNS involvement Hypoglycemia Ketoqcidosis ```
76
Cysteine synthesis pathway
Homocysteine + serine ——> cystathione By cysthationine synthase cystathionine——> a-ketobutyrate and cysteine By cystathionase
77
Cofactor needed for cysteine synthesis
PLP (pyridoxal phosphate )
78
Homocysteine synthesis pathway
Methionine ——> s Adenosylmethionine s Adenosylmethionine —-> a adenosylhomocysteine S adenosylhomocysteine—-> homocysteine
79
How to get from homocysteine to methionine
Add vit b12 + folic acid
80
Hypercysteinemja
Deficiency of cystathionine synthase Accumulation of homocysteine And remethylation causes high methionine levels
81
Hyperhomocysteinemoa symptoms
Mental retardation Dislocated lenses after age 3 Osteoporosis
82
Management of hyperhoomocysteinemia
Restrictions of methionine intake Feeding of beta une and pyridoxine Feeding of folate
83
What disease is high level homocysteine associated with
Mortality in coronary heart disease Dementia Alzeihmer disease
84
Methyl donor to homocysteine to obtain methionine
N5-methyltetrahydrofolate
85
Serine precursor
Glucose
86
Glycine precursors
Serine or threonine

Nutrition And Metabolism (17 decks)

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