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Biochem Final > Special Products > Flashcards

Special Products Flashcards

1
Q

What energy level are resting muscles in? What happens to creatine?

A

high ATP concentration, creatine is phosphorylated by creatine kinase»phosphocreatine

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2
Q

What energy level are exercising muscles in? What happens to creatine?

A

low ATP concentration, ATP is re-generated by creatine phosphate and ADP

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3
Q

What amino acids are needed to make creatine?

A

glycine and arginine

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4
Q

How does the body ensure there’s enough energy for continued muscle contraction?

A

uses phosphocreatine to re-generate ATP

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5
Q

How is creatiNINe made?

A

spontaneous cyclization of storage creatine

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6
Q

How is creatinine excreted?

A

proportional to muscle mass of the body

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7
Q

How can levels of creatinine be used to suspect kidney function impair?

A

sudden increase in creatinine usually is indicative of decreased kidney function

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8
Q

How do treatments of defect in creatine synthesis and defect in creatine uptake differ?

A

treatment for defect in creatine synthesis: creatine supplement
treatment for defect in creatine uptake: none

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9
Q
  • hypotonia (lack of muscle tone) but displays little skeletal or cardiac muscle pathology
  • also exhibit variety of neurological symptoms include epileptic seizures and intellectual disability
A

symptoms of both defects in creatine synthesis and uptake

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10
Q

What special products can be made from tyrosine?

A

catacholamines: DOPA, dopamine, norepinephrine and epinephrine
thyroid hormones: T3 and T4
melanin

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11
Q

How is DOPA made?

A

from tyrosine, catalyzed by tyrosine hydroxylase, requires co-factor THBtn

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12
Q

How is dopamine made?

A

from DOPA, catalyzed by DOPA decarboxylase

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13
Q

How is norepinephrine made?

A

from dopamine, catalyzed by dopamine beta-hydroxylase

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14
Q

How is epinephrine made?

A

from norepinephrine, catalyzed by norepinephrine N-methyltransferase

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15
Q

Where is dopamine made?

A

dopaminergic neurons

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16
Q

Where is norepinephrine made?

A

sympathetic neurons

17
Q

Where is epinephrine made?

A

adrenal medulla

18
Q

How is melanin made?

A

2 step process catalyzed by tyrosinase:

tyrosine»DOPA»Dopaquinone» either eumelanin or pheomelanine

19
Q
  • loss of function to tyrosinase caused by mutations to TYR gene
  • major complications include impaired vision and early appearance of skin tumors
A

Oculocutaneous albinism type I

20
Q

What disease is associated with dengeneration of dopaminergic neurons? What is the treatment?

A

Parkinson’s - can’t make dopamine

Treatement: administration of DOPA, relieves symptoms but not a cure

21
Q

What are the special products made from tryptophan?

A

serotonin, melatonin, niacin

tryptophan in turkey makes you sleepy and so does melatonin

22
Q

What enzyme is associated with the synthesis of thyroid hormones?

A

thyroid peroxidase

23
Q
  • contains more than 120 tyrosine residues

- small fraction of iodinated tyrosines can be coupled together

A

thyroglobulin

24
Q

How do the thyroid hormones T3 and T4 get released?

A

by the degradation of thyroglobulin in the lysosomes

25
Q

How is serotonin made?

A

hydroxylation and decarboxylation of tryptophan
Enzyme: tryptophan hydroxylase
Co-factor: THBtn

26
Q

Where is melatonin made?

A

pineal gland, released in dark cycle and induces sleepiness

27
Q

What is niacin made from?

A

tryptophan

Enzyme: IDO (anti-cancer drug target)

28
Q

The 3Ds and the ultimate D is associated with what disease? Due to what?

A

Pellagra - either due to lack of niacin or lack of tryptophan
(dermatitis, diarrhea, dementia, and death)

29
Q

Decarboxylation of glutamate

A

GABA, requires B6

30
Q

Decarboxylation of histidine

A

histamine, requires B6

31
Q
  • tripeptide with the sequence Glu-Cys-Gly
  • the bond between Glu and Cys is not a peptide bond, but a gamma-glutamyl linkage, which is very resistant to proteolysis
  • made in the liver
A

glutathione, the glutamyl linkage makes it a very stable molecule

32
Q

What’s the function of glutathione?

A

intracellular reducing agent:

  • scavenging free radicals
  • destroying peroxides
  • conjugate toxic molecules for excretion
33
Q

In order to re-generate glutathione, glutathione reductase needs to use what reducing agent?

A

NADPH

34
Q

How does G6PD deficiency affect glutathione regeneration?

A

NADPH is produced from G6PD in PPP

35
Q

What will B6 deficiency affect?

A
  • decarboxylation of glutamate (GABA) and histidine (histamine)
  • serine hydroxymethyltransferase (serine»glycine)
  • cystathione beta-synthase (homocysteine»cysteine)
  • all aminotransferases

Biochem Final (21 decks)

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