The KAL1 pVal610Ile mutation is a recessive mutation causing Kallmann syndrome

Fertil Steril. 2013 May;99(6):1720-3. doi: 10.1016/j.fertnstert.2013.01.099. Epub 2013 Feb 11.

Abstract

Objective: To present the clinical, genetic, biochemical, and molecular findings in two Chinese siblings with X-linked recessive Kallmann syndrome (KS).

Design: Case report.

Setting: University medical center.

Patient(s): Two Chinese siblings.

Intervention(s): Clinical evaluation, hormone assays, and gene mutation research.

Main outcome measure(s): Endocrinologic evaluation and genetic analysis.

Result(s): A missense mutation of KAL1, c.1828G>A, led to pVal610Ile substitution in two brothers with KS; their mother is heterozygous for this missense mutation encoded by single-nucleotide polymorphism rs2229013.

Conclusion(s): Mutation analysis revealed that a missense mutation of KAL1 in two brothers with KS, while their mother was heterozygous for this missense mutation encoded by the single-nucleotide polymorphism rs2229013. Variant alleles of KAL1 related to X-linked recessive KS expand the spectrum of KAL1 mutations causing KS.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Amino Acid Substitution / genetics*
  • Extracellular Matrix Proteins / genetics*
  • Female
  • Genes, Recessive / genetics*
  • Genetic Carrier Screening
  • Humans
  • Kallmann Syndrome / diagnosis
  • Kallmann Syndrome / genetics*
  • Male
  • Mutation, Missense / genetics
  • Nerve Tissue Proteins / genetics*
  • Pedigree
  • Young Adult

Substances

  • ANOS1 protein, human
  • Extracellular Matrix Proteins
  • Nerve Tissue Proteins