Goljan High Yield Biochemistry EC Flashcards Preview

ZBiochem > Goljan High Yield Biochemistry EC > Flashcards

Flashcards in Goljan High Yield Biochemistry EC Deck (43)
Loading flashcards...
1
Q

Rate limiting reaction in cholesterol synthesis

A

HMG Co reductase

2
Q

Findings in PKU

A

Mousy odor
Tyrosine missing hence it must be supplied in the diet
Can diagnose by amniocentesis and finding the abnormal gene
Eliminate phenylalanine from diet (no nutrasweet)

3
Q

I cell disease

A

Inability to phosphorylate the mannose residues of potential lysosomal enzymes in the golgi
Cannot be taken up by lysosomes to degrade complex substrates

4
Q

Number of glucoses necessary to build palmitic acid (16C compound)

A

4 glucoses

Each glucose produces 2 acetyl CoA w/ 2 carbons each

5
Q

Insulin lack in DKA

A

Decreased glycolysis, glycogenesis, fatty acid synthesis, storage of fat in adipose

6
Q

Uncoupling agents (alcohol, salicylates)

A

Produces brown fat from increased heat from reactions trying to increase the generation of more protons to make ATP

7
Q

Von Gierke’s

A

decreased glucose-6-phosphatase (gluconeogenic enzyme) with hypoglycemia and increase in glucose-6-phosphate with production of normal glycogen in the liver and kidneys
Stimulation tests with glucagon, fructose.
Cannot increase glucose levels owing to missing enzyme

8
Q

Biochemical processes in both cytosol and mitochondria

A

Urea cycle, heme synthesis, gluconeogenesis

9
Q

Female with pheochromocytoma, what dietary alteration?

A

Decrease phenylalanine and tyrosine in the diet

10
Q

Pregnant female with PKU, what diet?

A

Low phenylalanine
High tyrosine
(avoid neutrasweet)

11
Q

Lesch Nyhan

A

Absent HGPRT

Self-mutilation, hyperuricemia, mentalretardation

12
Q

Glucokinase

A

Only in the liver
High Vm and Km
Not inhibited by G6P

13
Q

Hexokinase

A

In all tissues
Low Vm and Km
Inhibited by G6P

14
Q

Branched chain amino acids/Maple syrup urine disease

A

Only muscle van metabolize maple syrup urine disease

15
Q

Key enzyme in gluconeogenesis

A

Fructose-1-6-bisphosphatase (catalyses conversion of F16BP to F6P)

16
Q

Locations of glucose-6-phosphatase (gluconeogenic hormone)

A

Liver, kidney, intestinal epithelium

17
Q

Shuttles and what they carry

A

Carnitine (even chained fatty acids)

Malate (NADH)

18
Q

Functions of LDL

A

Vit. D synthesis
Steroid synthesis
Cell membranes
Synthesis of bile salts

19
Q

Acetyl CoA uses

A

FA synthesis
CH synthesis
Ketone body synthesis
(NOT a substrate for gluconeogenesis)

20
Q

Urea cycle

A

Method of eliminating ammonia

Located in hepatocyte

21
Q

Epinephrine given and only small branches chains found

A

Debrancher deficiency

22
Q

Origin of apolipoproteins

A

apo-100 =Liver

apo-48=intestine

23
Q

Rate limiting step in glycogenolysis

A

glycogen phosphorylase

24
Q

Reason why liver can not use ketones for fuel

A

liver cannot activate acetoacetate in the mitochondria which requires succinyl CoA: Acetoacetate CoA transferase in order to convert AcAc into acetoacetyl CoA

25
Q

McArdles disease

A

absent muscle phosphorylase
Increased glycogen in muscle
No increase in lactic acid after exercise

26
Q

Pregnant woman is a beer drinker, what supplements does she need?

A

Folate (alcohol increases loss)
Fetal alcohol syndrome possible
(Irone is NOT affected by beer drinking)

27
Q

Insulin

A

Key hormone in fed state

28
Q

Glucagon

A

Key hormone in fasting state

29
Q

Mannose-6-phosphate

A

Involved in transfer of dolichol in the RER in the synthesis of O-linked glycosides

30
Q

Major source of NADPH

A

HMP shunt

Malate dehydrogenase reaction to a lesser extent

31
Q

Mutation changes an amino acid sequence, which one would have the greatest effect on migration in a serum protein electrophoresis?

A

One with the most negative charges (acidic)=Glutamine

Most basic=Arginine

32
Q

Mechanism of ketoacidosis in DKA

A

Increased beta oxidation of fatty acids and production of acetyl CoA (used by liver to synthesize ketone bodies)

33
Q

Promoter location

A

Linear gene drawing with labels, pick upstream locations

34
Q

Energy source for protein synthesis

A

GTP

35
Q

Isoenzyme with 2 genes, 4 subunits

A
LDH isoenzymes
LLLL
LLLH
LLHH
LHHH
HHHH
36
Q

Second messengers

A

ANP=cGMP
Insulin=TK
Nicotinic=ion channels

37
Q

Best method of detecting relatedness of new bacteria

A

Restriction fragment length polymorphism

38
Q

Enzyme used for PCR

A

DNA polymerase

39
Q

Source of glucose in a brain in starvation

A

Alanine (transaminated to pyruvate)

40
Q

Glutamine

A

Most abundant amino acid

Carries ammonia in nontoxic form

41
Q

Tyrosine

A

Thyroid hormones
Melanin
Neurotransmitters
Decreased in PKU

42
Q

Glycine

A

Inhibitory neurotransmitter
Blocked by tetanus toxin
Heme synthesis
Synthesis of bile acids

43
Q

Hepatic encephalopathy

A

Increase in aromatic amino acids (phenylalanine, tyrosine, tryptophan [PTT])
Increased synthesis of false neurotransmitters (GABA, octopamine) in hepatic encephalopathy
Branched chain amino acids inhibit synthesis of false neurotransmitters (given to Rx)