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Collective Deck > Biochemistry 2- Boards & Beyond > Flashcards

Biochemistry 2- Boards & Beyond Flashcards

1
Q

What amino acids do histones have a high content of

A

Lysine, arginine

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2
Q

What substitution occurs with sickle cell anemia

A

Polar glutamate for nonpolar valine

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3
Q

What are the 2 strictly ketogenic amino acids

A

Leucine, lysine

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4
Q

What is the cofactor for phenylalanine hydroxylase

A

BH4 (tetrahydrobiopterin)

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5
Q

What is the order of tyrosine metabolism

A

Tyrosine, DOPA, dopamine, norepinephrine, epinephrine

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6
Q

What enzyme is deficiency in oculocutaneous albinism

A

Tyrosinase

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7
Q

What is deficient in alkaptonuria

A

Homogentisic acid oxidase

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8
Q

What is the classic findings with alkaptonuria

A

Dark urine when left standing, arthritis, black pigment in cartilage/joints (calcification in IV discs), urine discoloration in children, dark pigment

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9
Q

What are common serotonin effects in carcinoid syndrome

A

Diarrhea (serotonin stimulates GI motility), increased fibroblast growth and fibrogenesis (leading to valvular lesions), flushing

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10
Q

What is the issue with Hartnup disease and how is it inherited

A

Absence of AA transport in proximal tubule; autosomal recessive

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11
Q

What are some common symptoms with Hartnu disease

A

Pellets (hyperpigmented rash, exposed areas of skin, red tongue, diarrhea, dementia)

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12
Q

What are the branched chain Amino acids

A

Valine, leucine, isoleucine

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13
Q

What is the deficiency in maple syrup urine disease and what are some symptoms

A

Deficiency of alpha keto acid dehydrogenase; increased branched chain AAs and alpha-ketoacids in plasma; neurotoxicity is main problem, urine smells sweet

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14
Q

What are some common symptoms of homocystinuria

A

Lens dislocation, long limbs, chest deformities, osteoporosis in childhood, blood clots, early atherosclerosis

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15
Q

What is the alanine cycle used fr

A

By the muscles to transfer nitrogen to liver

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16
Q

What is the rate limiting enzyme in the urea cycle

A

Carbamoyl phosphate synthetase I

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17
Q

What is the most common defect in urea cycle

A

Ornithine transcarbamylase

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18
Q

What is another name for vitamin B1

A

Thiamine

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19
Q

What is the term for thiamine deficiency not associated with alcohol and how does it present

A

Beriberi; dry has polyneuritis, muscle weakness; wet has tachycardia, high-output heart failure, edema

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20
Q

What is another name for vitamin B2

A

Riboflavin

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21
Q

What vitamin is required for synthesis of niacin from tryptophan

A

Vitamin B6

22
Q

What are the 4 Ds associated with niacin deficiency

A

Pellagra: dermatitis, diarrhea, dementia, death

23
Q

What is another name for vitamin B5 and what is it used in

A

Pantothenic acid; coenzyme A

24
Q

What is another name for vitamin B6

A

Pyridoxal phosphate

25
Q

What type of anemia can occur with vitamin B6 deficiency

A

Sideroblastic anemia (because iron cannot be incorporated into heme and so it accumulates in RBC cytoplasm)

26
Q

What is the only B vitamin with potential toxicity

A

B6

27
Q

What is biotin (vitamin B7) cofactor for

A

Carboxylation enzymes

28
Q

What vitamin is required for conversion of dopamine to norepinephrine

A

Vitamin C

29
Q

What vitamin can be in excess with sarcoidosis

A

Vitamin C

30
Q

What deficiency should you think of with impaired sexual development, poor wound healing, and loss of taste

A

Zinc

31
Q

What heredity are most lysosomal storage diseases

A

Autosomal recessive

32
Q

What is deficient in fabry’s disease, what accumulates

A

Alpha-galactosidase A is deficient; ceramics trihexoside accumulates

33
Q

What is the classic case seen with Fabry’s disease

A

Child with pain in hands/feet, lack of sweat, skin findings (angiokeratomas)

34
Q

What is deficient in gaucher disease and who is it most common in

A

Deficiency of glucocerebrosidase; ashkenazi Jews

35
Q

What should you think of with crinkled paper macrophages

A

Gaucher disease

36
Q

What is the common presentation of gaucher disease

A

Hepatosplenomegaly, severe bone pain, easy bruising from low platelets, join problems

37
Q

What is deficient with Niemann pick disease

A

Acid sphingomyelinase

38
Q

What is the classic case of Niemann pick disease

A

Previously well/healthy child, weakness/loss of motor skills, enlarged liver or spleen on physical exam, cherry red spot

39
Q

What are some conditions that commonly have cherry red spot

A

Niemann-pick, tay-Sachs, central retinal artery occlusion

40
Q

What is deficient with Krabbes disease

A

Galactocerebroside

41
Q

What is the classic case of Krabbes disease

A

Presents at 6 months, only neuro symptoms, progressive weakness, absent reflexes

42
Q

What is deficient with Tay Sachs and what accumulates

A

Deficiency of hexosaminidase A; GM2 ganglioside accumulates

43
Q

What is the presentation of Tay Sachs

A

Presents 3-6 months, weakness, exaggerates startle reaction, progresses to seizures/vision/hearing loss, cherry red spot, NO hepatosplenomegaly

44
Q

What is the classic Path finding with Tay Sachs

A

Lysosomes with onion skinning

45
Q

What is the deficiency with metaochromatic leukodystrophy

A

Arylsulfatase A

46
Q

What are common presentations of metachromatic leukodystrophy

A

Roughly 2 years old, ataxia, hypotonia

47
Q

What is deficient with Hurler’s syndrome and what accumulates

A

Deficiency of alpha-L-iduronidase; accumulation of heparin and dermatan sulfate

48
Q

What are some symptoms of hurler’s syndrome

A

Corneal clouding, coarse facial features, short stature, hepatosplenomegaly

49
Q

What is the heredity of hunters syndrome

A

X-linked recessive

50
Q

What is deficient with Hunter’s syndrome

A

Iduronate-2-sulfatase

51
Q

What makes hunter’s syndrome different from hurlers

A

No corneal clouding, later onset, behavioral problems, learning difficulty, trouble sitting still, often aggressive behavior

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