2.7.7.40: D-ribitol-5-phosphate cytidylyltransferase
This is an abbreviated version!
For detailed information about D-ribitol-5-phosphate cytidylyltransferase, go to the full flat file.
Word Map on EC 2.7.7.40
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2.7.7.40
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dystrophy
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muscular
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polysaccharide
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fukutin
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teichoic
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fukutin-related
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dystroglycanopathy
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glycan
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influenzae
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haemophilus
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walker-warburg
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dispensability
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malformation
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alpha-dystroglycan
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capsular
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hydrocephalus
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polyribitol
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lissencephaly
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o-mannose
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virulence-associated
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hypoplasia
- 2.7.7.40
-
dystrophy
-
muscular
- polysaccharide
-
fukutin
-
teichoic
-
fukutin-related
-
dystroglycanopathy
- glycan
- influenzae
-
haemophilus
-
walker-warburg
-
dispensability
-
malformation
- alpha-dystroglycan
- capsular
-
hydrocephalus
-
polyribitol
-
lissencephaly
-
o-mannose
-
virulence-associated
-
hypoplasia
Reaction
Synonyms
Bcs1, CDP-L-ribitol pyrophosphorylase A, CDP-ribitol (ribose) pyrophosphorylase, CDP-ribitol pyrophosphorylase, CDP-ribitol synthase, CDPribitol pyrophosphorylase, CRPPA, cytidine diphosphate ribitol pyrophosphorylase, cytidine diphosphate-l-ribitol pyrophosphorylase A, Cytidine diphosphoribitol pyrophosphorylase, cytidylyltransferase, cytidylyltransferase, ribitol 5-phosphate, isoprenoid synthase domain-containing protein, IspD, ribitol 5-phosphate cytidylyltransferase, TarI, TarIJ
ECTree
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General Information
General Information on EC 2.7.7.40 - D-ribitol-5-phosphate cytidylyltransferase
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malfunction
defects in DPM1/2/3 and ISPD are considered tertiary dystroglycanopathies since they synthesize the sugar donors Dol-P-Man (POMT1/2) and CDP-ribitol (presumably Fukutin and/or FKRP) for subsequent glycosyltransferases
metabolism
physiological function
ISPD is a CDP-ribitol (ribose) diphosphorylase that generates the reduced sugar nucleotide for the insertion of ribitol in a phosphodiester linkage to the glycoprotein, the enzyme is employed for the synthesis of the required sugar (alcohol) nucleotide needed for ribitol insertion into the M3 glycan
ISPD, similar to the DPM1/2/3 enzyme complex that generates dolichol-phosphomannose for initial mannosylation, generates CDP-ribitol for ribosylation of the phosphotrisaccharide. Both of these processes are involved in the generation of donors for the enzymes involved in functional glycosylation of alpha-dystroglycan and as such congenital muscular dystrophy (CMD) resulting from these enzymes can then be referred to as tertiary dystroglycanopathies
metabolism
CDP-ribitol pyrophosphorylase activity of the enzyme is required for correct alpha-dystroglycan glycosylation