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Test 23- BC Flashcards

(47 cards)

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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

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A

Converts glu-6 phosphate to glucose

*last step in production of glucose from gluconeogenesis or glycogenolysis

20
Q

AA w/ three titratable H

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

vit that helps w/ treatment of measles

22
Q

direction of DNA synthesis

23
Q

Activates trypsinogen to trypsin

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A

duodenal enteropeptidase

24
Q

most important enzyme for digestion of TG

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

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