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50 Cards in this Set

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  • Back
Under normal conditions brain derives energy from _ alone
Glucose
Major energy source for brain during starvation
Ketone bodies
_ 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
Vitamin B12 defficiency
Accumulation of glutamine results in _
Cerebral edema
Prolonged ammonia elevation causes _
Mental retardation
2 cell types of nervous system
Neurons and glia
2 major types of glial cells
Oligodendrocytes
Astrocytes
Produce myelin sheath
Oligodendrocytes
Provide metabolic support and protection for neurons
Astrocytes
Resemble macrophages - appear to protect against infection and remove cell debris
Microglia
Line ventricles and central canal of spinal cavity - involved in production, circulation and monitoring of CSF
Ependymal cells
Substances that can cross BBB
Caffeine, alcohol, nicotine, cocaine
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
Tryptophan
Serotonin and melatonin
Can brain absorb FA
NO
Energy in the brain is required for _
Transport process - maintenance of ion gradient and electrochemical potential across plasma membrane of neurons
Synthesis of neurotransmitters and neuromodulators (esp. peptides)
Certain things require glucose in addition to energy source - those are _
Production of Ach
Production of glutamate and GABA
Pentose phosphate pathway to provide NADPH (need for FA synthesis)
Spingolipids are derived from _
Alcohol sphingosine and contain ceramide
2 types of sphingolipids
Sphingomyelin
Glycosphingolipids
Difference of sphingomyelin and glycosphingolipids
Sphingomyelin doesnt contain sugar units
What accumulates in Niemann Pick disease
Sphingomyelin and cholesterol
Types A and B of Niemann Pick disease are due to defficiency of _
Sphingomyelinase
Type _ Niemann Pick disease is most common
A
Type C Niemann Pick disease is due to defect in _ and results in accumulation of _
Lipid transport
Cholesterol
In Gaucher disease what accumulates and what is the defficiency
Glucocerebrosidase accumulates due to deficiency in beta-glucosidase
Most common sphingolipidosis is _
Type I Gaucher disease
Types 1 and 3 are treatable by administration of _
Beta-glucosidase
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 _
Krabbes disease
In this disease ceramide trihexoside accumulates due to deficiency of alpha-galactosidase A - disease called _
Fabrys disease
In metachromatic leukodystrophy _ accumulates due to defficiency of _
Sulfatide - defficiency of lysosomal sulphatase
In TAY SACHS DISEASE - _ accumulates due to defficiency of _
GANGLIOSIDE GM2 - defficiency of HEXOSAMINIDASE A
Sphingolipidoses are autosomal recessive with exception of _ which is X linked
FABRYS
Only treatable sphingolipidoses currently are _
Gaucher and Fabrys
Patient presents with megaloblastic anemia and neurological deterioration - he has defficiency of _ also called _
B12 - pernicious anemia
Patient has defficiency of carbamoyl phosphate synthetase - disease is called _
Congenital hyperammonemia type I
Patient has defficiency of ornithine transcarbamoylase - most common urea cycle genetic defect - disease called _
Congenital hyperammonemia type II
Gaucher and Tay Sachs disease are more common in _
Ashkenazi Jews
In PKU enzyme that is defficient is _
Phenylalanine hydroxylase
6 year old male with progressive mental retardation, vomitting and hyperactivity - characteristic " musty" odor - diagnosis - treatment?
PKU
Diet modifications, tyrosine supplements
In starvation, gluconeogenesis depletes _ in liver, depletion inhibits TCA cycle and _ accumulates
Oxaloacetate
Acetyl CoA
Ketone bodies are produced ONLY in _
LIVER
Patient presents with hepatosplenomegaly, mental retardation and " crumpled tissue paper" cells - diagnosis
GAUCHER
Patient presents with mental retardation, blindness and characteristic cherry red spot on macula - diagnosis and prognosis
TAY SACHS - death in infancy
Patient presents with hepatosplenomegaly, mental retardation and cholesterol accumulation - diagnosis
Niemann Pick
Patient presents with skin rash and kidney failure - diagnosed with sphingolipidoses, patients brother and uncle have same disease - DX
Fabrys
Patient presents with mental retardation and absence of myelin - DX
Krabbes
DA, NE and EPI are made from _
Tyrosine
Serotonin and Melatonin are made from _
Tryptophan
Derived from histidine
Histamine
Purigenic NT include _
Adenosine
ATP
Dense core vesicles contain _
Peptides