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Flashcards in Metabolic Profile of the Brain Deck (56):
1

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

Glut 1 and Glut 3

2

what types of amino acids compete for transporter #4

phe, tyr, try and branched chain amino acids

3

serotonin and melatonin are made from

tryptophan

4

what inhibit the production of serotonin and melatonin

branch chain amino acids

5

phenylketonuria is a deficiency in

phenylalanine hydroxylase

6

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

melanin

7

what aa becomes essential in people with PKU

tyrosine

8

where are ketone bodies produced

liver

9

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

glucose

10

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

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

11

glutamate is made from

glucose via alpha ketogluturate

12

GABA is made from

glutamate

13

glutathionine is made from

glutamate

14

The Pentose phosphate pathway is essential for

antioxidant system and lipogenesis

15

composition of sphingolipids

sphingosine + fatty acid + X

16

glucose + ceramide

glucocerebroside

17

galactocerebroside

galactose + ceramide

18

what accumulates in gaucher disease

glucocrebroside

19

what accumulates in Krabbe's disease

galactocerebroside

20

ceramides containing a sulphated galactose

sulphatides

21

what accumulates in metachromatic leukodystrophy-MLD

suplphatides

22

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

gangliosides

23

what acccumulates in TaySach's diseasee

gangliosides (GM2)

24

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

hexosamindase A

25

what enzyme is deficient in fabry disease

alpha glactosidase A

26

what enzyme if deficient in Krabbe disese

beta galactosidase

27

what enzyme is deficieny in metacrhomatic leukodystrophy

arylsulfatase A

28

what enzyme is deficient in Niemann pick disease

sphingomyelinase

29

cherry red spot on the macula

tay-sach's disease

30

what is the substance that accumulates in gaucher disease

glucocerebroside

31

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

ganglioside Gm2

32

what is the substrate that accumulates in krabbe disease

glactocerebroside

33

what substances accumulates in fabry disease

ceramide trihexoside

34

what accumulates in niemann pick disease A and B

sphingomylein

35

what accumulates in niemann pick C and D

cholesterol

36

hepatosplenomegaly, metal retardation, crupled tissue paper appearance

gaucher disese

37

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

tay sachs

38

hepatosplenomegaly, metal retardation, cholesterol accumulation

niemann pick

39

skin rash, kidney failure

fabry disease

40

mental retradation, absence of myelin

krabbe disease

41

what sphingolipidoses is X linked

Fabry disease

42

what are the three types of gaucher disease

non-neuronopathic
acute neuronopathic
subacute neuronopathic

43

what form of gaucher disease affects infants

type 2

44

what type of gaucher is primary CNS disease

type 2

45

skeletal abnormalities is present in what type of gaucher

type 1 and type 3

46

reactions that use vitamin B12

convert homocysteine to methionine and to convert methylmalonly to succinyl coa

47

in vitamin b12 deficiency what accumulates in the urine

methyl malonate

48

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

by competitive inhibition of malonyl CoA carboxylase
by disrupting membrane structure through incorporation of methylmalonyl CoA producing branched chain fatty acids

49

how of ammonia reoved

by a glutamine synthetase reaction

50

the glutamate synthetase reaction converts

glutamate to gluamine

51

what does glutamine do to the mitochondria

increases mitochondrial permeability

52

removal of alpha ketogluturate from the TCA cycle results in

depletion of ATP

53

what other things also deplete when you ahve too much ammonia

glutamate and GABA

54

what are the two common causes of hyperammoniemia

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

55

type I urea cycle deficiency

carbomyl phosphate synthetase deficiency, lethal neonatal

56

type II urea cycle deficiency

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