disease 979 words KG: ent-dise-46e78f41 2026-03-19
kind:diseasesection:diseasesstate:published
Contents

Woodhouse-Sakati Syndrome (WSS)

Disease Info
PrevalenceExtremely rare; <100 cases reported worldwide
InheritanceAutosomal recessive - requires two mutant alleles
Age of onsetChildhood to early adulthood
Ethnic distributionHigher prevalence in Middle Eastern populations (consanguinity)
Sex distributionEqual males and females
Common mutationsN145S, R325W, splice site mutations
FunctionInvolved in ubiquitination and protein degradation pathways
PathogenesisLoss of DCAF8 function leads to accumulation of yet-unidentified substrate proteins
HypogonadismPrimary gonadal failure, delayed or absent puberty
Diabetes mellitusType 2 diabetes, insulin resistance
HypothyroidismPrimary hypothyroidism in some cases
Growth hormone deficiencyShort stature in childhood
DatabasesOMIMOrphanetClinicalTrialsPubMed

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