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Neuroanatomy > Metabolic Profile of the Brain > Flashcards

Metabolic Profile of the Brain Flashcards

1
Q

what are the GLUT transporters responsible for bringing glucose into the brain ?

A

Glut 1 and Glut 3

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2
Q

what types of amino acids compete for transporter #4

A

phe, tyr, try and branched chain amino acids

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3
Q

serotonin and melatonin are made from

A

tryptophan

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4
Q

what inhibit the production of serotonin and melatonin

A

branch chain amino acids

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5
Q

phenylketonuria is a deficiency in

A

phenylalanine hydroxylase

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6
Q

people with PKU disease often have decreased _______ synthesis resulting in a deficiency in pigmentation

A

melanin

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7
Q

what aa becomes essential in people with PKU

A

tyrosine

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8
Q

where are ketone bodies produced

A

liver

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9
Q

what is the main energy source when the body is in acute hypoglycemia (24 hours starvation)

A

glucose

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10
Q

what is the main energy source for prolonged hypoglycemia (starvation)

A

liver produces ketone bodies that take over for glucose and serve as the major energy source thus sparing muscle protein

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11
Q

glutamate is made from

A

glucose via alpha ketogluturate

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12
Q

GABA is made from

A

glutamate

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13
Q

glutathionine is made from

A

glutamate

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14
Q

The Pentose phosphate pathway is essential for

A

antioxidant system and lipogenesis

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15
Q

composition of sphingolipids

A

sphingosine + fatty acid + X

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16
Q

glucose + ceramide

A

glucocerebroside

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17
Q

galactocerebroside

A

galactose + ceramide

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18
Q

what accumulates in gaucher disease

A

glucocrebroside

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19
Q

what accumulates in Krabbe’s disease

A

galactocerebroside

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20
Q

ceramides containing a sulphated galactose

A

sulphatides

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21
Q

what accumulates in metachromatic leukodystrophy-MLD

A

suplphatides

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22
Q

ceramide oligosaccharides containing one or more mols of NANA (sialic acid)

A

gangliosides

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23
Q

what acccumulates in TaySach’s diseasee

A

gangliosides (GM2)

24
Q

what is the enzyme is deficiency in tay sach’s disese

A

hexosamindase A

25
Q

what enzyme is deficient in fabry disease

A

alpha glactosidase A

26
Q

what enzyme if deficient in Krabbe disese

A

beta galactosidase

27
Q

what enzyme is deficieny in metacrhomatic leukodystrophy

A

arylsulfatase A

28
Q

what enzyme is deficient in Niemann pick disease

A

sphingomyelinase

29
Q

cherry red spot on the macula

A

tay-sach’s disease

30
Q

what is the substance that accumulates in gaucher disease

A

glucocerebroside

31
Q

what is the substrate that accumulates in tay sach’s disease

A

ganglioside Gm2

32
Q

what is the substrate that accumulates in krabbe disease

A

glactocerebroside

33
Q

what substances accumulates in fabry disease

A

ceramide trihexoside

34
Q

what accumulates in niemann pick disease A and B

A

sphingomylein

35
Q

what accumulates in niemann pick C and D

A

cholesterol

36
Q

hepatosplenomegaly, metal retardation, crupled tissue paper appearance

A

gaucher disese

37
Q

metal retardation, blindness, death in infancy, cherry red spot on macula

A

tay sachs

38
Q

hepatosplenomegaly, metal retardation, cholesterol accumulation

A

niemann pick

39
Q

skin rash, kidney failure

A

fabry disease

40
Q

mental retradation, absence of myelin

A

krabbe disease

41
Q

what sphingolipidoses is X linked

A

Fabry disease

42
Q

what are the three types of gaucher disease

A

non-neuronopathic
acute neuronopathic
subacute neuronopathic

43
Q

what form of gaucher disease affects infants

A

type 2

44
Q

what type of gaucher is primary CNS disease

A

type 2

45
Q

skeletal abnormalities is present in what type of gaucher

A

type 1 and type 3

46
Q

reactions that use vitamin B12

A

convert homocysteine to methionine and to convert methylmalonly to succinyl coa

47
Q

in vitamin b12 deficiency what accumulates in the urine

A

methyl malonate

48
Q

how does the accumulation of methylmalonyl CoA interfere with myelin sheath formation

A

by competitive inhibition of malonyl CoA carboxylase

by disrupting membrane structure through incorporation of methylmalonyl CoA producing branched chain fatty acids

49
Q

how of ammonia reoved

A

by a glutamine synthetase reaction

50
Q

the glutamate synthetase reaction converts

A

glutamate to gluamine

51
Q

what does glutamine do to the mitochondria

A

increases mitochondrial permeability

52
Q

removal of alpha ketogluturate from the TCA cycle results in

A

depletion of ATP

53
Q

what other things also deplete when you ahve too much ammonia

A

glutamate and GABA

54
Q

what are the two common causes of hyperammoniemia

A

acquired hyperammonemia (liver disease) and hereditary hyperammonemia (genetic deficiency of a urea cycle enzyme)

55
Q

type I urea cycle deficiency

A

carbomyl phosphate synthetase deficiency, lethal neonatal

56
Q

type II urea cycle deficiency

A

ornithine transcarbamoylase deficiency, X linked, most common, treat with arginigne and low protien diet

Neuroanatomy (9 decks)

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