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HOXB6 and Hypospadias

In a study screening four candidate genes in 90 unrelated Chinese patients with hypospadias, rare heterozygous variants were identified, including a missense change in HOXB6 (c.367T>C (p.Cys123Arg)) that was absent from 380 control chromosomes (PMID:17003840). Although the overall number of HOXB6-specific variants is low, the detection in more than one proband provides modest genetic evidence for its involvement, despite the absence of segregation data from affected relatives.

A subsequent large-scale candidate gene association study also reported single nucleotide polymorphisms in HOXB6 among other developmental genes associated with hypospadias (PMID:23727413). In the absence of direct functional or experimental evidence for HOXB6, the current data rely on in silico predictions and the conservation of altered amino acids. Key take‑home: Although the evidence remains limited, HOXB6 should be considered a potential candidate for hypospadias risk, meriting further functional validation to support its clinical utility.

References

  • European Journal of Human Genetics • 2007 • Mutation screening of BMP4, BMP7, HOXA4 and HOXB6 genes in Chinese patients with hypospadias PMID:17003840
  • The Journal of Urology • 2013 • Hypospadias and genes related to genital tubercle and early urethral development PMID:23727413

Evidence Based Scoring (AI generated)

Gene–Disease Association

Limited

HOXB6 variants were identified in a limited number of cases (2 variants in 90 probands) without segregation data (PMID:17003840), supported by additional SNP association evidence (PMID:23727413).

Genetic Evidence

Limited

Two heterozygous missense variants in HOXB6, notably c.367T>C (p.Cys123Arg), were reported in a case series, suggesting a tentative genetic association with hypospadias, though larger cohorts and segregation analysis are needed.

Functional Evidence

Limited

No direct functional studies for HOXB6 have been provided; current evidence relies on in silico predictions and conservation data without experimental validation.