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BIOCHEMISTRY > SPHINGO copy 2 > Flashcards

SPHINGO copy 2 Flashcards

1
Q

enzyme in the reaction to produce 3 ketodihydrosphinganine

A

serine palmitoyl transferase

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2
Q

enzyme to produce sphinganine

A

3 ketosphinganine reductase

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3
Q

Precursor of ceramide

A

Sphinganine

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4
Q

Difference between Glycosphingolipids from sphingomyelin

A

Glycosphingolipids do not contain phosphate and polar head function is provided by a monosaccharide or oligosaccharide attached directly to the ceramide by an O-glycosidic bond

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5
Q

Cermaide reacts with what compound to form glucocerebroside

A

UDP-glucose

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6
Q

Site of glycosphingolipid synthesis

A

Golgi apparatus

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7
Q

the acid in the sphingomyelin in the gray mater of the brain

A

stearic acid

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8
Q

Functions of Gangliosides

A
  1. Component of ganglion cells of the CNS (nerve endings. 2. Component of cholera toxin responsible for sever diarrhea in cholera 3. Bind immune system cells to sites of injury in the inflammatory response.
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9
Q

Five monosaccharides linked to ceramide

A

One glucoe, two galactose, one n-acetylgalactosamine and one nacetylneuraminic acid

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10
Q

Phospholipase present in mammalian tissues and pancreatic juice

A

Phospholipase A2

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11
Q

Carbon atom Phosolipase C acts on

A

Carbon 3

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12
Q

What does Phospholipase release when it acts on the glycerol backbone?

A

Phosphorylated alcohol

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13
Q

Site of where you can find Phospholipase C

A

liver lysosomes

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14
Q

What does phospholipase A1 cleave from the glycerol backbone?

A

Fatty acid

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15
Q

Phospholipase that releases an alcohol attached to the phosphate group of a phosphoglycerol molecule

A

Phospholipase D

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16
Q

Enzyme that degrades sphingomyelin to form a ceramide

A

sphingomyelinase

17
Q

Sphingomyelinase is an example of what phospholipase

A

Phospholipase C

18
Q

What is left behind when Ceramide is cleaved by ceramidase

A

Sphingosine and a free fatty acid

19
Q

What enzyme cleaves Ceramide?

A

Ceramidase

20
Q

Disease where in there is an absence of ceramidase

A

Farber’s Disease

21
Q

Disease where in there is an accumulation of sphingomyelin due to absence of sphingomyelinase

A

Niemann Pick Disease

22
Q

Disease where in there is an absence of B-galactosidase and accumulation of galactocerebroside

A

Krabbe’s disease

23
Q

Disease where in there is an accumulation of ganglioside

A

Generalized Gangliosidosis

24
Q

Disease where in there is an accumulation of GM2

A

Tay-Sach’s disease

25
Q

Disease where in there is an accumulation of globoside

A

Fabry’s disease

26
Q

Disease where in there is an accumulation of glucocerebroside

A

Gaucher’s disease

27
Q

deficient enzyme in Tay-Sach’s disease

A

ß-N-acetylhexosaminidase

BIOCHEMISTRY (6 decks)

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