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Flashcards in Test 23- BC Deck (47):
1

Glycogen degradation in skeletal muscle increases several hundred fold after the onset of contraction. This is due to enzyme activation by which substance?

Ca

2

What couples glycogen degradation to skeletal muscle contraction?

Calcium mediated myophosphorylase activation

Increased Ca in the cytosol activates phosphorylase kinase>
activates muscle phosphorylase

3

Maple syrup urine disease

defect in branched chain alpha ket acid DH>
inability to break down (LIV) beyond their deaminated alpha keto acid state>
elevated levels lead to neurotoxicity

4

Dystonia (sustained muscle contractions), poor feeding, maple syrup scent of urine

MSUD

5

Tx for MSUD

restrict branched chain A

6

Krebs cycle mneumonic

Citrate
is = isocitrate
krebs= alpha keto
starting= succinyl coa
substrate= succinate
for
making
oxaloacteate

7

Branched chain AA

leucine
isoleucine
valine

8

Restrict in pts w/ PKU

phenylalanine

9

Defective breakdown of tyrosine

alkaptonuria

10

What is the major AA responsible for transferring N to the liver for disposal?

alanine

11

What happens to amino groups during protein catabolism?

Transferred to alpha ketoglutarate to form GLUTAMATE

12

What happens to glutamate generated from catabolism of proteins?

Glutamate is processed in the liver to form urea which is disposed of

13

malate, citrate, OAA

intermediates of TCA cycle

14

L- citrulline

UREA cycle

AA produced as intermediate in conversion of ornithine to arginosuccinate during urea sycle

15

What forms calactitol?

excess circulating galactose in galactosemia by Aldose reductase

16

converts UDP galactose to UDP glucose

UDP galactose 4 epimerase

17

Beta galctosidase def

leads to accumulation of glycosaminoglycans (keratin sulfate in lysosomes)

18

def in aldolase B

hereditary frustose inolerance

19

Glucose 6 phosphatase

Converts glu-6 phosphate to glucose


*last step in production of glucose from gluconeogenesis or glycogenolysis

20

AA w/ three titratable H

Histidine
arginine
lysine
aspartic acid
glutamic acid
cysteine
tyrosine

21

vit that helps w/ treatment of measles

vit A

22

direction of DNA synthesis

5' to 3'

23

Activates trypsinogen to trypsin

duodenal enteropeptidase

24

most important enzyme for digestion of TG

lipase

25

condition that causes lipase def and leads to poor fat absorption and steatorrhea

chronic pancreatitis

26

activates pepsinogen to pepsin

acidic pH and small amt of pre-existing pepsin

27

pancreatic amylase

hydrolyzes complex carbs to oligo, di and monosaccharides

28

secreted in response to LOW duodenal pH

secretin>
stimulates bicarb secretion form pancrease and gallbladder>
reduced gastrin secretion/stomach acid

29

Sxs of vit deficiency years after dietary abscence.

can ONLY occur with vit B12

30

Enzymes in heme biosynthesis pathway inactivated by lead

zinc contaiing gamm-aminolevulinate dehydratase and ferrochelatase

31

What accumulates in lead poisoning?

gama-ALA
protoporphyrin IX accumulate>

32

How does lead poisoning cause anemia?

inhibition of enzymes in heme pathway>

decreased production of heme>
microcytic anemia d/t a lack of Hb

33

Rate limiting step in heme synthesis that uses B6 and is inhibited by increased heme levels

ALA synthase

34

Defect in porphogilinogen deaminase

Acute intermittent porphyria

35

Acute attacks of abdominal pain and neuropsychiatrix sxs

Urine is dark on exposure to light

Acute intermittent porphyria

36

Defect in uroporphyrinogen decarboxylase

porphyria cutanea tarda

37

Ab to citrullinated peptides

Rheumatoid Arthritis

38

Anti-centromere Abs

CREST

39

Antibodies to DS DNA

SLE

40

Autoantibodies that target Fc portion of human IgG

RF

41

anti-nuclear antibodies

nonspecific for connective tissue disorders

42

Antiphospholipid abs

SLE and antiphospholipid syndrome

43

DNA dep RNA polymerase that incorporates short RNA primers into replicating DNA

Primase

44

synthesizes daughter DNA strands

DNA II

45

replaces RNA primers w/ DNA segments

DNA I

46

Cofactor for synthesis of tyrosine, dopa and 5HT

BH4

47

Precursor for 5HT

tryptophan