Test 23- BC Flashcards

1
Q

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

A

Ca

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2
Q

What couples glycogen degradation to skeletal muscle contraction?

A

Calcium mediated myophosphorylase activation

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

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3
Q

Maple syrup urine disease

A

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

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4
Q

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

A

MSUD

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5
Q

Tx for MSUD

A

restrict branched chain A

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6
Q

Krebs cycle mneumonic

A
Citrate
is = isocitrate
krebs= alpha keto
starting= succinyl coa
substrate= succinate
for
making
oxaloacteate
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7
Q

Branched chain AA

A

leucine
isoleucine
valine

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8
Q

Restrict in pts w/ PKU

A

phenylalanine

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9
Q

Defective breakdown of tyrosine

A

alkaptonuria

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10
Q

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

A

alanine

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11
Q

What happens to amino groups during protein catabolism?

A

Transferred to alpha ketoglutarate to form GLUTAMATE

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12
Q

What happens to glutamate generated from catabolism of proteins?

A

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

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13
Q

malate, citrate, OAA

A

intermediates of TCA cycle

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14
Q

L- citrulline

A

UREA cycle

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

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15
Q

What forms calactitol?

A

excess circulating galactose in galactosemia by Aldose reductase

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16
Q

converts UDP galactose to UDP glucose

A

UDP galactose 4 epimerase

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17
Q

Beta galctosidase def

A

leads to accumulation of glycosaminoglycans (keratin sulfate in lysosomes)

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18
Q

def in aldolase B

A

hereditary frustose inolerance

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19
Q

Glucose 6 phosphatase

A

Converts glu-6 phosphate to glucose

*last step in production of glucose from gluconeogenesis or glycogenolysis

20
Q

AA w/ three titratable H

A
Histidine
arginine
lysine
aspartic acid
glutamic acid
cysteine
tyrosine
21
Q

vit that helps w/ treatment of measles

A

vit A

22
Q

direction of DNA synthesis

A

5’ to 3’

23
Q

Activates trypsinogen to trypsin

A

duodenal enteropeptidase

24
Q

most important enzyme for digestion of TG

A

lipase

25
Q

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

A

chronic pancreatitis

26
Q

activates pepsinogen to pepsin

A

acidic pH and small amt of pre-existing pepsin

27
Q

pancreatic amylase

A

hydrolyzes complex carbs to oligo, di and monosaccharides

28
Q

secreted in response to LOW duodenal pH

A

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

29
Q

Sxs of vit deficiency years after dietary abscence.

A

can ONLY occur with vit B12

30
Q

Enzymes in heme biosynthesis pathway inactivated by lead

A

zinc contaiing gamm-aminolevulinate dehydratase and ferrochelatase

31
Q

What accumulates in lead poisoning?

A

gama-ALA

protoporphyrin IX accumulate>

32
Q

How does lead poisoning cause anemia?

A

inhibition of enzymes in heme pathway>

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

33
Q

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

A

ALA synthase

34
Q

Defect in porphogilinogen deaminase

A

Acute intermittent porphyria

35
Q

Acute attacks of abdominal pain and neuropsychiatrix sxs

Urine is dark on exposure to light

A

Acute intermittent porphyria

36
Q

Defect in uroporphyrinogen decarboxylase

A

porphyria cutanea tarda

37
Q

Ab to citrullinated peptides

A

Rheumatoid Arthritis

38
Q

Anti-centromere Abs

A

CREST

39
Q

Antibodies to DS DNA

A

SLE

40
Q

Autoantibodies that target Fc portion of human IgG

A

RF

41
Q

anti-nuclear antibodies

A

nonspecific for connective tissue disorders

42
Q

Antiphospholipid abs

A

SLE and antiphospholipid syndrome

43
Q

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

A

Primase

44
Q

synthesizes daughter DNA strands

A

DNA II

45
Q

replaces RNA primers w/ DNA segments

A

DNA I

46
Q

Cofactor for synthesis of tyrosine, dopa and 5HT

A

BH4

47
Q

Precursor for 5HT

A

tryptophan