Following is the structural formula of ganglioside GM, a macromolecular gly- colipid (meaning that it contains lipid and monosaccharide units joined by glycosidic bonds). (b) Но CH,OH (d) HO CH,OH (f) NH CH,OH OH (a) C=0- OCH,CHCHCH=CH(CH,)12CH3 OH ОН Но. ČH3 (c) NHC(CH,) 16CH3 (e) НО ОН OH НООС NH HO C=O CH3 Ganglioside GM or Tay-Sachs ganglioside In normal cells, this and other gangliosides are synthesized continuously and degraded by lysosomes, which are cell organelles containing digestive enzymes. If pathways for the degradation of gangliosides are inhibited, the gangliosides accumulate in the cen- tral nervous system, causing all sorts of life-threatening consequences. In inherited diseases of ganglioside metabolism, death usually occurs at an early age. Diseases of ganglioside metabolism include Gaucher's disease, Niemann-Pick disease, and Tay- Sachs disease. Tay-Sachs disease is a hereditary defect that is transmitted as an au- tosomal recessive gene. The concentration of ganglioside GM, is abnormally high in this disease because the enzyme responsible for catalyzing the hydrolysis of glycosidic bond (b) is absent.
Following is the structural formula of ganglioside GM, a macromolecular gly- colipid (meaning that it contains lipid and monosaccharide units joined by glycosidic bonds). (b) Но CH,OH (d) HO CH,OH (f) NH CH,OH OH (a) C=0- OCH,CHCHCH=CH(CH,)12CH3 OH ОН Но. ČH3 (c) NHC(CH,) 16CH3 (e) НО ОН OH НООС NH HO C=O CH3 Ganglioside GM or Tay-Sachs ganglioside In normal cells, this and other gangliosides are synthesized continuously and degraded by lysosomes, which are cell organelles containing digestive enzymes. If pathways for the degradation of gangliosides are inhibited, the gangliosides accumulate in the cen- tral nervous system, causing all sorts of life-threatening consequences. In inherited diseases of ganglioside metabolism, death usually occurs at an early age. Diseases of ganglioside metabolism include Gaucher's disease, Niemann-Pick disease, and Tay- Sachs disease. Tay-Sachs disease is a hereditary defect that is transmitted as an au- tosomal recessive gene. The concentration of ganglioside GM, is abnormally high in this disease because the enzyme responsible for catalyzing the hydrolysis of glycosidic bond (b) is absent.
Biochemistry
9th Edition
ISBN:9781319114671
Author:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.
Publisher:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.
Chapter1: Biochemistry: An Evolving Science
Section: Chapter Questions
Problem 1P
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Describe this glycosidic bond.
Transcribed Image Text:Following is the structural formula of ganglioside GM, a macromolecular gly-
colipid (meaning that it contains lipid and monosaccharide units joined by
glycosidic bonds).
(b)
Но
CH,OH
(d)
HO
CH,OH
(f)
NH
CH,OH
OH
(a)
C=0-
OCH,CHCHCH=CH(CH,)12CH3
OH
ОН
Но.
ČH3
(c)
NHC(CH,) 16CH3
(e)
НО ОН
OH
НООС
NH
HO
C=O
CH3
Ganglioside GM or Tay-Sachs ganglioside
In normal cells, this and other gangliosides are synthesized continuously and degraded
by lysosomes, which are cell organelles containing digestive enzymes. If pathways for
the degradation of gangliosides are inhibited, the gangliosides accumulate in the cen-
tral nervous system, causing all sorts of life-threatening consequences. In inherited
diseases of ganglioside metabolism, death usually occurs at an early age. Diseases of
ganglioside metabolism include Gaucher's disease, Niemann-Pick disease, and Tay-
Sachs disease. Tay-Sachs disease is a hereditary defect that is transmitted as an au-
tosomal recessive gene. The concentration of ganglioside GM, is abnormally high in
this disease because the enzyme responsible for catalyzing the hydrolysis of glycosidic
bond (b) is absent.
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