Ribose-5-phosphate isomerase

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Ribose-5-phosphate isomerase
Properties of human protein
Mass / length primary structure 311 amino acids
Identifier
Gene name RPIA
External IDs
Enzyme classification
EC, category 5.3.1.6 isomerase
Response type Rearrangement
Substrate D-ribulose-5- (di, tri) phosphate
Products D-ribose-5- (di, tri) phosphate
Occurrence
Homology family RPI
Parent taxon Creature
Orthologue
human House mouse
Entrez 22934 19895
Ensemble ENSG00000153574 ENSMUSG00000053604
UniProt P49247 P47968
Refseq (mRNA) NM_144563 NM_009075
Refseq (protein) NP_653164 NP_033101
Gene locus Chr 2: 88.69 - 88.75 Mb Chr 6: 70.77 - 70.79 Mb
PubMed search 22934 19895

Ribose-5-phosphate isomerase (RPI) is the enzyme that converts ribose-5-phosphate and ribulose-5-phosphate into one another. This reaction equilibrium is part of the pentose phosphate pathway in almost all living things and in the assimilation of carbon dioxide in plants. A lack of RPI by mutations in RPIA - gene in humans is rare and causes leukodystrophy and peripheral neuropathies due to accumulated ribitol and arabitol .

Catalyzed reaction equilibrium

Ru5P    R5P

Ribulose-5-phosphate is rearranged to ribose-5-phosphate and vice versa. The di- and triphosphates are also accepted as substrates.

Individual evidence

  1. UniProt P49247
  2. Huck JH, Verhoeven NM, Struys EA, Salomons GS, Jakobs C, van der Knaap MS: Ribose-5-phosphate isomerase deficiency: new inborn error in the pentose phosphate pathway associated with a slowly progressive leukoencephalopathy . In: Am. J. Hum. Genet. . 74, No. 4, April 2004, pp. 745-51. doi : 10.1086 / 383204 . PMID 14988808 . PMC 1181951 (free full text).
  3. EC  5.3.1.6

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