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USMLE BIOCHEMISTRY > Aminoacids metabolism and Diseases > Flashcards

Aminoacids metabolism and Diseases Flashcards

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USMLE® Step 1 flashcards
1
Q

What is the variable portion in Aminoacids

A

Side Chain R (Especific for each aminoacid)

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2
Q

What is the constant portion in Aminoacids

A

Amino group + Carboxylate group (Same for all)

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3
Q

How many essential aminoacids are there?

A

9

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4
Q

Essential aminoacides

A

Arginine
Histidine
Leucine
Isoleucine
Lysine
Methionine
Penylalaline
Threonie
Tryptophan
Valine

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5
Q

Which aminoacids are essential Ketogenic

A

Leucine / Lysine

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6
Q

How do you categorize aminoacids

A

Whether if they can be degraded to glucose or ketone bodies

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7
Q

What is the main function of Urea Cycle

A

Conversion of toxic ammonia into non-toxic UREA

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8
Q

Ammonia is the product of

A

Aminoacid Metabolism (Amino group)

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9
Q

Urea cycle’s reactant

A

Ammonia and CO

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10
Q

Urea cycle’s final product

A
  • Ornithine
  • Urea
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11
Q

Which molecules are necessary in order to convert Ammonia into Urea

A

ATP and Aspartate

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12
Q

Where does the Urea cycle occur

A

Mitochondria and Cytosol of Liver Cells

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13
Q

Regulation of Urea Cycle

A

Allosteric at Carbamoyl Phosphate Synthetase-1
Stimulated by : N-acetylglutamine

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14
Q

Ornithine Transcarbamylase deficiency causes stoppage at which step in Urea Cycle

A

Last step

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15
Q

Ornithine Transcarbamylase deficiency inheritance pattern

A

X-Linked Recessive

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16
Q

Lab findings in Ornithine Transcarbamylase deficiency

A

Increased Ammonia and OROTIC ACID
Decreased BUN

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17
Q

What is the Cahill Cycle function?

A

Transport of amino groups from AA in muscle into the liver for disposal of AA via Urea Cycle

18
Q

Which via does Cahill Cycle send AA from muscle to Liver

A

Alanine

19
Q

Cori Cycle function

A

Transport lactate made in muscle into the liver for conversion of Glucose via Gluconeogenesis

20
Q

PKU is caused by deficiency of

A

Phenylalaline Hydroxilase

21
Q

Increased and decreased products in PKU

A

Increased Phenylalanine
Decreased Tyrosine

22
Q

PKU clinical features

A

Intellectual disability/Seizures + ALBINISM + MUSTY ODDOR + Eczema

23
Q

Diagnosis of PKU can be made by

A

Detection of Excess Phenyl Ketones in urine

24
Q

Treatment of PKU

A

Avoidance of Phenylalanine
Supplementation with Tyrosine

25
Q

Maple Syrup Disease is caused by deficiency of

A

Branched-chain Alpha Ketoacid Dehydrogenase

26
Q

Which AA are branched-chain

A

Valine, Leucine, Isoleucine.

27
Q

Treatment of Maple Syrup Disease

A

Avoidance of Branched-chain aminoacids

28
Q

Maple Syrup disease clinical features

A

Fruity urine smell + Intelectual Disability + Vomiting + Seizures

29
Q

Alkaptonuria is caused by deficiency of

A

Homogentisate oxidase

30
Q

Alkaptonuria clinical features

A

Arthritis + Blue discoloration of Ear/Sclera/Cheeks

31
Q

Alkaptonuria main feature for diagnosis

A

Urine turns black when exposed to air

32
Q

Homocystinuria is caused by deficiency of

A

Cysthatione Synthase or Metionine Shyntase

33
Q

Homocystinuria’s clinical features

A

MARFANOID HABITUS + LENS SUBLUXATION + Atherosclerotic disease + Intelectual disability

34
Q

Diagnosis of Homocystinuria

A

Elevated Homocysteine in urine

35
Q

Cysthatione Synthase needs ___ as cofactor

A

B6

36
Q

Methionine Synthase needs ____ as Cofactor

A

B12

37
Q

Treatment of Homocystinuria

A

MS deficiency = Supplementation with Methionine
CS deficiency = Avoidance of methionine and Supplementation with B6

38
Q

Propionic Acidemia is caused by deficiency of

A

Propionyl Co-A Carboxylase

39
Q

Propionic Acidemia clinical features

A

Poor feedin + Lethargy + Cardiomyopathy

40
Q

Diagnosis of Propionic Acidemia

A

Elevated levels of
- Ammonia
- Propionyl Co-A
- Propionic acid

in URINE/BLOOD

USMLE BIOCHEMISTRY (17 decks)

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