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March 2020 > Copper bio > Flashcards

Copper bio Flashcards

1
Q

AA affinity of Cu+

A

Cysteine and Methionine

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2
Q

AA affinity of Cu2+

A

Aspartic Acid, Glutamic Acid, Histidine

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3
Q

Principle which explains why Copper can displace other 1st row transition metals in complexes

A

Irving-Williams series

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4
Q

Two cuproporteins which shuttle to the nucleus and act as TFs

A

MTF1 and ATOX1

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5
Q

binding element with which MTF1 interacts

A

MRE

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6
Q

chaperone delivering Cu to the P-type ATPases

A

ATOX1 antioxidant 1 copper chaperone

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7
Q

cuproprotein responsible for upregulating cyclin D and promoting S phase entry

A

ATOX1 antioxidant 1 copper chaperone

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8
Q

A treatment for Wilsons disease, Ammonium tetrathiomolybdate complexes with ____ which has unknown consequences

A

ATOX1 antioxidant 1 copper chaperone

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9
Q

Regulates copper homeostasis by interacting with 26S proteasome

A

CCS Copper Chaperone for Superoxide dismutase

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10
Q

CCS is a copper chaperone for ___

A

superoxide dismutase SOD1

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11
Q

cuproproteins localized to the golgi membrane, rich in thioles

A

metallothioneins MTs

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12
Q

Copper importer mutated in Huppke Brendal syndrome

A

SLC33A1 (COPT1) copper transporter 1

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13
Q

mediates ATP7A/B translocation in response to Cu changes, which is disrupted in Huppke Brendal

A

SLC33A1 (COPT1) copper transporter 1

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14
Q

3 TMEM domain proteins which localize to apical side of epithelial cells to help absorb copper

A

SLC33A1/2 (COPT1/2)

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15
Q

Cupric enzyme in catecholamine synthesis pathway

A

Dopamine Beta Monooxygenase DBH

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16
Q

Dopamine beta monooxygenase uses what cofactor

A

ascorbic acid

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17
Q

amine oxidases using copper

A

LOX AOC1-3

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18
Q

redox cofactor of amine oxidases

A

topaquinone TPQ

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19
Q

cupric vascular adhesion protein (also enzyme)

A

AOC3 amine oxidase copper containing 3

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20
Q

main enzyme responsible for digestion of ingested histamine

A

AOC1 amine oxidase copper containing 1

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21
Q

cupric enzyme participating in decidualization of endometrium before implantaion

A

AOC1 amine oxidase copper containing 1

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22
Q

cupric enzyme targeted by mAbs to treat immune disease

A

AOC3 amine oxidase copper containing 3

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23
Q

cupric enzyme cleaved by BMP-1 in the extracellular space in order to mature

A

LOX lysyl oxidase

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24
Q

LOX function

A

crosslinks ECM proteins

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25
Q

cupric enzyme inhibiting keratinocyte differentiation

A

LOXL2 lysyl oxidase like 2

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26
Q

large TMEM cuproprotein at the last steps of the respiratory chain

A

COX CcO cytochrome c oxidase

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27
Q

3 subunits mtDNA the rest nuclear

A

COX CcO cytochrome c oxidase

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28
Q

converts 2O2- into H202 and O2

A

SOD superoxide dismutase

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29
Q

cupric enzyme preventing ROS in the extracellular fraction

A

SOD3 superoxide dismutase 3

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30
Q

cuproprotein which causes crosslinking by oxidizing phenolic substrates

A

laccase LACC1

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31
Q

cuproprotein complexed with fatty acid synthase on peroxisomes

A

Laccase LACC1

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32
Q

cuproprotein regulating the bioenergetic state of macrophages

A

Laccase LACC1

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33
Q

crosslinked by LOX

A

collagen and elastin

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34
Q

LOX converts ___ into highly reactive ___

A

lysines into highly reactive aldehydes

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35
Q

LOX inhibitor

A

beta aminopopionitrile BAPN

36
Q

major Cu chaperone in the circulation

A

Ceruloplasmin CP

37
Q

a decrease of this serum Cuproprotein is diagnostic of wilson’s disease

A

Ceruloplasmin CP

38
Q

number of Cu on Ceruloplasmin CP

A

6

39
Q

in serum, oxidizes Fe to Fe(III) allowing transferrin to carry it

A

Ceruloplasmin CP

40
Q

Serum levels decrease in Cu deficiency, menkes disease or wilsons disease

A

Ceruloplasmin CP

41
Q

Mechanism by which CP is decreased in Cu deficiency

A

fast turnover of apoprotein

42
Q

transmembrane ferroxidase transporting Fe from enterocytes into circulation

A

Hephaestin HEPH

43
Q

rate-limiting enzyme of melanin synthesis, Cu protein

A

Tyrosinase

44
Q

single TMEM, glycoprotein coordinating 2 Cu, hydroxylates phenols and converts to quinone

A

Tyrosinase

45
Q

Organ which doesn’t express ATP7A

A

Liver

46
Q

ATP7A protects against excitotoxicity by releasing intracellular Cu stores in response to ______ activation

A

N-methyl-d-aspartate receptor NMDAR

47
Q

Phenotypic effects of COX dysfunction

A

intracerebral lactic acidosis; CNS degeneration; Ataxia

48
Q

Phenotypic effects of dopamine beta hydroxylase dysfunction

A

hypothermia, hypotension, diarrhea

49
Q

Phenotypic effects of lysyl oxidase LOX dysfunction

A

hematoma, emphysema, petechiae, arterial defects

50
Q

Phenotypic effects of sulfhydryl oxidase QSOX1/2 dysfunction

A

Bladder diverticula, hernia, hair abnormalities

51
Q

Phenotypic effects of superoxide dismutase SOD dysfunction

A

CNS degeneration

52
Q

organs with the highest copper concentrations in healthy adults

A

Liver, kidney

53
Q

amount of copper in the system of a healthy adult

A

110mg

54
Q

percentage of ingested copper normally absorbed

A

30-40%

55
Q

Year and location of Menkes original publication

A

1962 at Columbia University in New York

56
Q

Australian who discovered copper’s role in MD

A

David Danks

57
Q

David Danks’ findings that led him to hypothesize copper for MD

A

Similarity of hair to Cu-deficient wool; Cu buildup in duodenal mucosa

58
Q

Cu enzyme responsible for keratin crosslinking

A

QSOX1/2 sulfhydryl oxidase

59
Q

three proteins carrying copper in circulation

A

ceruloplasmin, transcuperin, albumin

60
Q

organ which does not express ATP7A

A

liver

61
Q

localization of ATP7A in most polarized cell types

A

basolateral

62
Q

localization of ATP7A in choroid plexus epithelium

A

apical

63
Q

What ATP7A complexes with in the CSF

A

Albumin and histidine

64
Q

two names of alternative receptor (besides CTR/Slc33A1) delivering copper into the cell

A

divalent metal transporter DMT1; SLC11A2

65
Q

caused by ATP7A defects in the absence of systemic copper issues

A

distal motor neuropathy

66
Q

number of exons in human ATP7A

A

23

67
Q

total size of ATP7A gene

A

140kb

68
Q

Where ATP7A diverges from homology with ATP7B

A

5’ third of gene

69
Q

approximate number of AA in ATP7A protein

A

1500

70
Q

percentage breakdowns of different types of ATP7A mutations identified in patients

A

22% indels, 22% duplications, 17% missense, 18% nonsense, 18% splicing defects

71
Q

region of ATP7A most often pathogenically mutated

A

exon 7-10

72
Q

2 ATP7A mutations amenable to early copper treatment

A

Gly666Arg; Gly727Arg

73
Q

Common bone issues of MD

A

pectus excavatum, pectus carinatum, wormian bones in the skull, osteopenia, spontaneous fracture of long bones

74
Q

age of onset for neurological regression in MD

A

6-8 weeks

75
Q

seizure phenotype of early stage MD

A

focal seizures progressing into status epilepticus

76
Q

Seizure phenotype of intermediate stage MD

A

epileptic spasms including west syndrome; hypsarrhythmia

77
Q

Seizure phenotype of late stage MD

A

multifocal, tonic spasms, myoclonus

78
Q

MD phenotype in gray matter

A

neuron loss and gliosis

79
Q

MD phenotype in basal ganglia

A

cystic infarction

80
Q

MD phenotype in cerebellum

A

Purkinje cell loss

81
Q

vascular abnormalities in cerebral arteries of MD

A

thin walled, erratic, tortuous

82
Q

defect bringing most OHS patients into clinic

A

bladder diverticula

83
Q

Low to borderline normal intellectual ability, muscular hypotonia, bladder diverticula

A

OHS

84
Q

name of zebrafish ATP7A mutant line

A

calamity

85
Q

mouse lines with ATP7A mutations surviving until early postnatal development

A

viable-brindled, blotchy

March 2020 (2 decks)

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