SORD

Chr 15

sorbitol dehydrogenase

Also known as: HEL-S-95n, HMNR8, RDH, SDH, SORD1, SORDD, XDH

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]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtNeuronopathy, distal hereditary motor, autosomal recessive 8

Clinical highlights

Gene-disease validity (ClinGen)
Charcot-Marie-Tooth disease · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
41
Pubs (1 yr)
P/LP submissions
P/LP missense
1.27
LOEUF
Multiple*
Mechanism· predicted
📖
GeneReview available — SORD
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.27LOEUF
pLI 0.000
Z-score 0.74
OE 0.80 (0.521.27)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.35Z-score
OE missense 1.08 (0.951.22)
185 obs / 172.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.80 (0.521.27)
00.351.4
Missense OE?1.08 (0.951.22)
00.61.4
Synonymous OE?1.09
01.21.6
LoF obs/exp: 13 / 16.2Missense obs/exp: 185 / 172.0Syn Z: -0.59

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

SORD · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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