A.A. + urea cycle Flashcards

1
Q

Ketogenic:
Glucogenic:
Both:

A
  • Lys, Leu
  • Ala, ser, glu, asp
  • Trp, Ile
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2
Q

Improper intake of E.A.A.

A

Protein energy malnutrition

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3
Q
  • Phase 1 of protein digestion occurs in _
  • Enzymes involved
  • Pepsinogen > pepsin via ?
A
  • Stomach
  • Gastrin, pepsin
  • HCL
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4
Q
  • Phase 2 of protein digestion occurs in _
  • Enzymes involved
  • Trypsinogen > trypsin via?
A
  • Intestine
  • Pancreozymin/cholecystokinin, secretin, trypsin
  • Enterokinase
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5
Q

Deficiency of pancreatic secretion

A

Steatorrhea(Fatty poop)

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6
Q

A.A. absorption occurs in _

A

Intestine

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7
Q

A.A transport across serosal membranes is

A

Bidirectional

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8
Q

GSH should be high is which organs?
A.A. absorption via?
Transports A.A as _

A
  • RBC, intestine, kidney
  • Y-glutamyl cycle
  • Dipeptides
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9
Q

GSH synthase deficiency

A
  • Oxoprolinuria(hemolytic anemia)
  • y-glu-cys accumulates
  • Oxoproline excreted in urine
  • Less GSH > oxidative stress
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10
Q

Proteases help recognize what protein to degrade. 2 types and their subtypes.

A
  • Lysosomal: Cathepsins
  • Cytosolic: Calpains, metalloproteinases
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11
Q

PEST sequences

A
  • Proteins with short half life
  • Pro, glu, ser, thr
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12
Q

D-oxidases

A
  • Irreversible
  • In peroxisomes of liver, kidney
  • Uses FAD, FMN
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13
Q

Glutamate DH

A
  • In mitochondria
  • Reversible
  • Glu > @KG + NH3
  • Forward by NAD, backwards by NADPH
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14
Q

T/F: If NH3 is high, glutamate DH favors glutamate formation

A

True(backwards rxn)

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15
Q

Non-ox deamination:
Which A.A.?

A
  • Enamine > imino > @ketoacid + NH3
  • Ser, thr, cys
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16
Q

What forms the enamine?
Is it reversible?
Serine > ? and Thr > ?

A
  • Serine/Threonine dehydratase(B6-requiring)
  • No
  • Serine > pyruvate and Thr > @ketobutyrate
17
Q

Deamination of side chain amides

A
  • Irreversible
  • Gln > Glu + NH3 glutaminase
  • Asn > Asp + NH3 asparginase
18
Q

Transamination

A
  • Reversible
  • needs B6
  • No NH3 production
  • SGOT, SGPT, AST, ALT
19
Q

Aminotransferases for most A.A are in _

A

Liver

20
Q

Detox of NH3 > urea in _

A

Liver

21
Q

Urea in circulation is filtered by _

A

Kidney

22
Q

Blood high in urea?
Blood high in NH3?

A
  • Kidney problem
  • Liver problem
23
Q

Muscle transports NH3 as _
Brain releases NH3 as _

A
  • Alanine
  • Glutamine(carries 2 NH3)
24
Q

T/F: All reactions of the urea cycle occur in the mitochondria

A

False. Rxns 1,2 in mitochondria matrix while rxns 3,4,5 in cytoplasm

25
Q

RDS of urea cycle

A

CPS-1

26
Q

Urea cycle needs 3 ATP and 1 PP. How does it compensate for this?

A

By Glutamate DH rxn and Fumarate > OXAC

27
Q

Acetyl CoA + glutamate > NAG via _
NAG signals _ and _

A
  • NAG synthase
  • CO2 and NH3
28
Q

Defect of urea cycle leads to _

A

Hyperammonemia

29
Q

CPS-1 deficient:
OTC deficient:

A
  • low carbamoyl phosphate
  • high carbamoyl phosphate, orotic acid