Neuroscience Biochemistry

Front 1

Under normal conditions brain derives energy from _ alone

Back 1

Glucose

Front 2

Major energy source for brain during starvation

Back 2

Ketone bodies

Front 3

_ causes inhibition of normal FA synthesis and increase in odd-chain fatty acids in brain tissues and decreased synthesis of methionine required for methylation of nucleic acids

Back 3

Vitamin B12 defficiency

Front 4

Accumulation of glutamine results in _

Back 4

Cerebral edema

Front 5

Prolonged ammonia elevation causes _

Back 5

Mental retardation

Front 6

2 cell types of nervous system

Back 6

Neurons and glia

Front 7

2 major types of glial cells

Back 7

Oligodendrocytes
Astrocytes

Front 8

Produce myelin sheath

Back 8

Oligodendrocytes

Front 9

Provide metabolic support and protection for neurons

Back 9

Astrocytes

Front 10

Resemble macrophages - appear to protect against infection and remove cell debris

Back 10

Microglia

Front 11

Line ventricles and central canal of spinal cavity - involved in production, circulation and monitoring of CSF

Back 11

Ependymal cells

Front 12

Substances that can cross BBB

Back 12

Caffeine, alcohol, nicotine, cocaine

Front 13

Drink for athletes contain branched chain amino acids that compete with _ for aa transporter in the brain. These decreases _ and _ production which are precursors for tiredness, relaxation and sleep

Back 13

Tryptophan
Serotonin and melatonin

Front 14

Can brain absorb FA

Back 14

NO

Front 15

Energy in the brain is required for _

Back 15

Transport process - maintenance of ion gradient and electrochemical potential across plasma membrane of neurons
Synthesis of neurotransmitters and neuromodulators (esp. peptides)

Front 16

Certain things require glucose in addition to energy source - those are _

Back 16

Production of Ach
Production of glutamate and GABA
Pentose phosphate pathway to provide NADPH (need for FA synthesis)

Front 17

Spingolipids are derived from _

Back 17

Alcohol sphingosine and contain ceramide

Front 18

2 types of sphingolipids

Back 18

Sphingomyelin
Glycosphingolipids

Front 19

Difference of sphingomyelin and glycosphingolipids

Back 19

Sphingomyelin doesnt contain sugar units

Front 20

What accumulates in Niemann Pick disease

Back 20

Sphingomyelin and cholesterol

Front 21

Types A and B of Niemann Pick disease are due to defficiency of _

Back 21

Sphingomyelinase

Front 22

Type _ Niemann Pick disease is most common

Back 22

A

Front 23

Type C Niemann Pick disease is due to defect in _ and results in accumulation of _

Back 23

Lipid transport
Cholesterol

Front 24

In Gaucher disease what accumulates and what is the defficiency

Back 24

Glucocerebrosidase accumulates due to deficiency in beta-glucosidase

Front 25

Most common sphingolipidosis is _

Back 25

Type I Gaucher disease

Front 26

Types 1 and 3 are treatable by administration of _

Back 26

Beta-glucosidase

Front 27

Galactocerebroside accumulates due to defficiency of beta-galactosidase, characterized by demyelination. Macrophages aggregate forming multinucleated cells called globoid cells around blood vessels. This disease is called _

Back 27

Krabbes disease

Front 28

In this disease ceramide trihexoside accumulates due to deficiency of alpha-galactosidase A - disease called _

Back 28

Fabrys disease

Front 29

In metachromatic leukodystrophy _ accumulates due to defficiency of _

Back 29

Sulfatide - defficiency of lysosomal sulphatase

Front 30

In TAY SACHS DISEASE - _ accumulates due to defficiency of _

Back 30

GANGLIOSIDE GM2 - defficiency of HEXOSAMINIDASE A

Front 31

Sphingolipidoses are autosomal recessive with exception of _ which is X linked

Back 31

FABRYS

Front 32

Only treatable sphingolipidoses currently are _

Back 32

Gaucher and Fabrys

Front 33

Patient presents with megaloblastic anemia and neurological deterioration - he has defficiency of _ also called _

Back 33

B12 - pernicious anemia

Front 34

Patient has defficiency of carbamoyl phosphate synthetase - disease is called _

Back 34

Congenital hyperammonemia type I

Front 35

Patient has defficiency of ornithine transcarbamoylase - most common urea cycle genetic defect - disease called _

Back 35

Congenital hyperammonemia type II

Front 36

Gaucher and Tay Sachs disease are more common in _

Back 36

Ashkenazi Jews

Front 37

In PKU enzyme that is defficient is _

Back 37

Phenylalanine hydroxylase

Front 38

6 year old male with progressive mental retardation, vomitting and hyperactivity - characteristic " musty" odor - diagnosis - treatment?

Back 38

PKU
Diet modifications, tyrosine supplements

Front 39

In starvation, gluconeogenesis depletes _ in liver, depletion inhibits TCA cycle and _ accumulates

Back 39

Oxaloacetate
Acetyl CoA

Front 40

Ketone bodies are produced ONLY in _

Back 40

LIVER

Front 41

Patient presents with hepatosplenomegaly, mental retardation and " crumpled tissue paper" cells - diagnosis

Back 41

GAUCHER

Front 42

Patient presents with mental retardation, blindness and characteristic cherry red spot on macula - diagnosis and prognosis

Back 42

TAY SACHS - death in infancy

Front 43

Patient presents with hepatosplenomegaly, mental retardation and cholesterol accumulation - diagnosis

Back 43

Niemann Pick

Front 44

Patient presents with skin rash and kidney failure - diagnosed with sphingolipidoses, patients brother and uncle have same disease - DX

Back 44

Fabrys

Front 45

Patient presents with mental retardation and absence of myelin - DX

Back 45

Krabbes

Front 46

DA, NE and EPI are made from _

Back 46

Tyrosine

Front 47

Serotonin and Melatonin are made from _

Back 47

Tryptophan

Front 48

Derived from histidine

Back 48

Histamine

Front 49

Purigenic NT include _

Back 49

Adenosine
ATP

Front 50

Dense core vesicles contain _

Back 50

Peptides