SORD

Sorbitol dehydrogenase (SORD; EC 1.1.1.14) catalyzes the interconversion of polyols and their corresponding ketoses, and together with aldose reductase (ALDR1; MIM 103880), makes up the sorbitol pathway that is believed to play an important role in the development of diabetic complications (summarized by Carr and Markham, 1995 [PubMed 8535074]). The first reaction of the pathway (also called the polyol pathway) is the reduction of glucose to sorbitol by ALDR1 with NADPH as the cofactor. SORD then oxidizes the sorbitol to fructose using NAD(+) cofactor.[supplied by OMIM, Jul 2010]

Sorbitol Dehydrogenase

Polyol dehydrogenase that catalyzes the reversible NAD+-dependent oxidation of various sugar alcohols. Is mostly active with D-sorbitol (D-glucitol), L-threitol, xylitol and ribitol as substrates, leading to the C2-oxidized products D-fructose, L-erythrulose, D-xylulose, and D-ribulose, respectively (PubMed:3365415).
Is a key enzyme in the polyol pathway that interconverts glucose and fructose via sorbitol, which constitutes an important alternate route for glucose metabolism. The polyol pathway is believed to be involved in the etiology of diabetic complications, such as diabetic neuropathy and retinopathy, induced by hyperglycemia (PubMed:12962626, PubMed:29966615, PubMed:25105142).
May play a role in sperm motility by using sorbitol as an alternative energy source for sperm motility (PubMed:16278369).
May have a more general function in the metabolism of secondary alcohols since it also catalyzes the stereospecific oxidation of (2R,3R)-2,3-butanediol. To a lesser extent, can also oxidize L-arabinitol, galactitol and D-mannitol and glycerol in vitro. Oxidizes neither ethanol nor other primary alcohols. Cannot use NADP+ as the electron acceptor (PubMed:3365415).

Biological Process flagellated sperm motilityISS:UniProtKB
Biological Process fructose biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process glucose metabolic processManual Assertion Based On ExperimentTAS:UniProtKB
Biological Process glucuronate catabolic process to xylulose 5-phosphateIEA:Ensembl
Biological Process L-xylitol catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process L-xylitol metabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process response to cadmium ionIEA:Ensembl
Biological Process response to copper ionIEA:Ensembl
Biological Process response to hormoneIEA:Ensembl
Biological Process response to nutrient levelsIEA:Ensembl
Biological Process response to osmotic stressIEA:Ensembl
Biological Process response to xenobiotic stimulusIEA:Ensembl
Biological Process sorbitol catabolic processManual Assertion Based On ExperimentIDA:UniProtKB

Mitochondrion membrane
Cell projection, cilium, flagellum
Associated with mitochondria of the midpiece and near the plasma membrane in the principal piece of the flagellum. Also found in the epididymosome, secreted by the epididymal epithelium and that transfers proteins from the epididymal fluid to the sperm surface.

Sorbitol dehydrogenase deficiency with peripheral neuropathy (SORDD):
An autosomal recessive disorder characterized by motor axonal neuropathy, slowly progressive distal muscle weakness mainly affecting the lower limbs, difficulty walking, and increased serum sorbitol. Additional variable features are distal sensory impairment, upper limb tremor, scoliosis, and mild hearing loss.