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Medicine Sem 1 > Biochemistry week 15 > Flashcards

Biochemistry week 15 Flashcards

(47 cards)

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1
Q

Tryptophan metabolism results in (3)

A
  • Niacin, serotonin , melatonin
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2
Q

Histidine metabolism results in

A
  • Histamine
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3
Q

Glycine metabolism results in

A
  • Heme
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4
Q

Arginine metabolism results in (3)

A

Creatine , urea , nitric oxide

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5
Q

Glutamate metabolism results in

A

GABA

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6
Q

What are the 20 amino acids broken down into

A
  • Intermediates in carbohydrates and lipid metabloism
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7
Q

Name the amino acid that form ocaloacetate

A
  • Aspartate through transamination
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8
Q

Name the amino acids that form a ketoglutarate via glutamate(4)

A
  • Glutamine
    -Proline
    -Arginine
    -Histidine
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9
Q

Name the amino acids that form pyruvate(5)

A
  • Alanine, serine,Glycine,Cysteine, Threonine
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10
Q

Name the amino acids that form fumarate and the process

A
  • Phenylalanine and Tyrosine
  • PAH (phenylalanine hydroxylase ) catalyzes phenylalanine hydroxylation producing tyrosine
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11
Q

What type of amino acids are Phenylalanine and Tyrosine

A
  • Glucogenic and ketogenix
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12
Q

What is PhenylKetonuria caused by

A

-Deficiency of phenylalanine hydroxylase activity due to:
-Defective enzyme
-Deficient BH4 cofactor

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13
Q

Name the three Elevated Phenylalanine metabolites

A

-Phenylpyruvate ,
-phenylacetate, phenyllactate

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14
Q

What are the effects of Phenylketonuria on the central nervous system

A

-Severe intellectual disability
-Developmental delay, -microcephaly,
-seizures

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15
Q

What are the effetcs of Phenylketonuria in relation to hypopigmentation

A

Leads to :
-Fair hair, light skin, blue eyes due to decreased melanin synthesis

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16
Q

What is used to detect elevated phenyalanine blood levels

A
  • Laboratory testing
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17
Q

What is the function of the enzyme phenylalanaine hydroxylase

A
  • converts phenylalanine to tyrosine
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18
Q

What is Albinism

A
  • Genetic disorder affecting tryosine metabolism
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19
Q

What does Albinism lead to

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A
  • Mealanin deficiency, causing partial of full lack of pigment in skin, hair, eyes
20
Q

What are the symptoms of Albinism

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A
  • Hypopigmentation of skin, hair and eyes
    -Vison defects
  • Increased risk of skin cancer due lack of melanin production
21
Q

What is Parkinsons disease caused by

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A

-Dgeneration and depigmentation of neurons in the substantia nigra

22
Q

What is the parkinson disease lead to

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A
  • Dopamine deficiency, affecting volutaary muscle movement
23
Q

What is Methionine

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A

Sulfur containing amino acid

24
Q

What is the role of Methionine

Study These Flashcards
A

It is converted to SAM, a major methyl donor
and also produces:
-Hcy(Homosysteine, which is linked to thrombosis

25
Name the steps in Methionine metabolsim
- SAM synthesis: Methionine condenses with ATP to form SAM -Methyl Transfer: SAM donates methyl group forming SAH - SAH hydrolysis: SAH is hydrolysed to Hcy and Adenosine
26
To regenerate methionine to maintain SAm what is reuqired
- Folic acid and vitamin B12
27
Name the amino acids that produce succinyl CoA
- Valine and isoleucine - Threonine
28
Explain how valine and isoleucine produces succinyl CoA - What is required
They are generated into propionyl CoA , then converted into methylmalonyl CoA and then to succinyl CoA - Biotin and vitamin B12
29
How is threonine converted to succinyl CoA
-Dehydrated to a ketobutyrate , then converted itno propionyl then succinyl CoA
30
Name the 6 amino acids that form acetyl CoA or AcetoacetylCoA
- Tryptophan -Leucine -Isoleucine -lysine -Phenylalanine - Tyrosine
31
Name whether these amino acids are Ketogenic or Glucogenic: -Tryptophan -Leucine -Isoleucine -Lysine
-Tryptophan: Glucogenic , Ketogenic -Leucine: Ketogenic -Isoleucine: Ketogenic and glucogenic -Lysine: Ketogenic
32
What amino acids are involved in Branched chain Amino Acid degredation: - What does it require(5) -What does it produce
-Isoleucine, Leucine,Valine - Thiamine pyrophosphate, lipoic acid, FAD, NAD+ and CoA - produce NADH
33
What are the end of the products of Catabolism -Isoelcuine -Valine - Leucine
- Acetyl CoA and succinyl CoA - Succinyl CoA -Acetoacetate and Acteyl CoA
34
What is Maple syrup Urine disease
Autosomal recessive disorder
35
What is Maple Syrup Urine disease caused by
- Deficiency in branched -chain aketo acid dehydrogenase
36
What are the symptoms of Maple Syrup urine disease
- Male syrup like odor of urine -Intellectual disability
37
How is maple syrup urine disease treated
- With a Synthetic formula free of BCAA( Branche chain a keto acid dehydrogenase
38
In relation to the maple syrup urine disease what is the symptoms of the accumaltion of the branched chain a keto acid dehyrogenase (BCAA) in the blood
- Vomitting -Neuological problems -Ketoacidosis
39
What is the active form of folic acid and where is it produced
-Tetrahydrofolate(THF) -Produced from folate by dihydrofolate reductase in a 2 step reaction
40
What is the function of Tetrahydrofolate (THF)
Carries one-carbon units
41
Explain what happens in glutamine synthesis
- It converts glutamate to glutamine
42
How is serine formed
3-phosphoglycerate or from glycine via SHMT
43
What is glycine formed from
Serine via SHMT
44
What is cysteine formed from
-From Hcy (homocysteine) and serine, which is hydrolysed to cysteine and a ketobutyrate
45
What causes homocystinuria
Defect in cystathionine B -synthase
46
Give some characteristcs of homocystinuria
- Long limbs -Lens dislocation -Intellectual disbility
47
What does cystinuria cause
- Kidney stones

Medicine Sem 1 (61 decks)

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