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Biochemistry > Proteins > Flashcards

Proteins Flashcards

1
Q

Deficient enzyme in MSUD and accumulating AA?

A

Branched chain alpha ketoacid dehydrogenase

Accumulation of valine, leucine and isoleucine

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2
Q

Enzyme deficient in phenylketonuria?

A

Phenylalanine hydroxylase.

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3
Q

Derivatives of tryptophan

A

Serotonin, niacin, melatonin

Has largest side chain

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4
Q

Precursor of homocysteine

A

Methionine

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5
Q

Only imino acid

Contributed to collagen structure

A

Proline

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6
Q

Phenylalanine derivatives

A 
Phenylalanine
Tyrosine
L-dopa
Dopamine
Norepinephrine 
Epinephrine
Thyroxine
Melanin
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7
Q

Test for folic acid deficiency

A

N- formiminoglutamate excretion test

After ingestion of large amounts of histidine, individuals lacking folic acid will excrete increased amounts of FIGIu in urine.

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8
Q

Essential amino acids?

A

PVT TIM HALL always ARGues, never TYRes

Phenylalanine
Valine
Tryptophan
Threonine
Isoleucine
Methionine
Histidine*
Arginine*
Leucine
Lysine
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9
Q

Conditionally non-essential AA?

A

Arginine

Histidine

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10
Q

Usual configuration of peptide bonds?

A

Trans configuration

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11
Q

Secondary protein structures include

A

Alpha helix

Beta sheets

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12
Q

Combination of adjacent secondary structures?

A

Motifs or super secondary structures

Ex. B-a-B units, hair pin, meander, barrel

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13
Q

Number of residues per turn in alpha helical?

A

3.6 AA per turn

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14
Q

Aggregation of motifs

A

Domain (tertiary structure)

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15
Q

Characterized by spongiform changes, astrocytic gliomas and neuronal loss secondary to deposition of insoluble protein aggregates in neural cells

A

Prion diseases
PrPsc accumulation

Creutzfeld Jacob disease
Kuru
Scrapie
Bovine spongiform encephalopathy

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16
Q

What protein accumulates in Alzheimer’s disease?

A

Beta amyloid forming senile plaques and neurofibrillary tangles

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17
Q

Heme is composed of:

A

Protoporphyrin IX

Ferrous iron Fe 2+

18
Q

Major inhibitory NT in spinal cord

Smallest amino acid

A

Glycine

19
Q

Hemoglobin form that binds oxygen?

A

Relaxed form (not taut form)

20
Q

AA residue in myoglobin and hemoglobin that plays a role in O2 binding

A

Histidine

21
Q

Differentiate hemoglobin and myoglobin curves

A

Hemoglobin: binds 4 O2 molecules, sigmoidal curve

Myoglobin: hyperbolic curve, only 1 O2 molecule

22
Q

Factors that cause shift to the right of HgB curve

A

CABET do the right thing, LET GO

CO2
Acidity
BPG
Exercise
Temperature
23
Q

Characteristics of methemoglobin

A

Oxidized for of Hb (Fe3+)
Cyanosis, anxiety, headache, dyspnea
Chocolate cyanosis

24
Q

Treatment for methemoglobin emit

A

Oral methylene blue, ascot if acid

IV methylene blue

25
Q

Has 200x affinity for Hb

Causes cherry pink color with excess exposure

A

Carbon monoxide

Carboxyhemoglobinemia

26
Q

Mutations in hereditary sperocytosis

Diagnostic test?

Treatment?

A

Shape of RBC becomes spherical
Mutations in ankyrin, spectrum, band 3, band 4.1
Anemia, splenomegaly, jaundice

Osmotic fragility test
Splenectomy

27
Q

AA substitution in sickle cell disease

A

Glutamate -> valine

28
Q

Medication that increases levels of fetal hemoglobin

A

Hydroxyurea

29
Q

Substitution in hemoglobin C

A

Lysine (+) to glutamate (-)

30
Q

Mutation in alpha thalassemia

Spectrum of presentation?

A

Alpha chains

Silent carrier, a thalassemia trait, HbH disease, hydrops fetalis

31
Q

Spectrum of B thalassemia presentation?

A

B thalassemia minor

B thalassemia major

32
Q

Most abundant protein in the body

A

Collagen

33
Q

Structure of collagen

A

Triple helix of alpha chains
Gly-X-Y
X- proline
Y- hydroxyproline and hydroxylysine

34
Q

Collagen type affected in Ehler Danlos syndrome?

Presentation?

A

Type III collagen

Hyper mobile joints
Hyper extensible skin
Tendency to bleed
Increased risk of berry aneurysms

35
Q

Deficiency in type I collagen

A

Osteogenesis imperfecta

Multiple fractures
Blue sclerae
Hearing loss
Dental imperfections

36
Q

Defect in scurvy

A

Inability to hydroxylate Y component of collagen due to vit C deficiency
Decreased collagen cross linking

37
Q

Defect in Alport syndrome?

Presentation?

A

Type IV collagen

SNHL + hematuria, ESRD, RF

38
Q

Characterized by kinky hair and growth retardation

Dietary deficiency in copper causing inability to form cross links that strengthen collagen fibers

A

Menke’s syndrome

39
Q

Collagen defect in Epidermolysis bullish

A

Type VII collagen

40
Q

Inheritance pattern of Marfan’s syndrome

Gene defect?

Cardiac manifestation?

A

AD

Fibrillin gene

Ascending aortic dilation and dissection

41
Q

Component present in collagen but absent in elastin

A

Hydroxylysine

Minimal hydroxyproline

Biochemistry (5 decks)

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