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Partial chromosome Y deletion

MedGen UID:
267211
Concept ID:
C1507149
Cell or Molecular Dysfunction
Synonyms: Partial deletion of chromosome Y; Partial deletion of Y; Y chromosome deletions; Y chromosome microdeletions; Y-chromosome microdeletions
SNOMED CT: Partial chromosome Y deletion (717158001)
Modes of inheritance:
Y-linked inheritance
MedGen UID:
163055
Concept ID:
C0814045
Genetic Function
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on the Y chromosome.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0015607
Orphanet: ORPHA1646

Definition

A severe deficiency of spermatogenesis. Chromosome Y deletions are a frequent genetic cause of male infertility. The mode of transmission follows a Y-linked pattern, with incomplete penetrance, but as deletions are often associated with infertility, they generally occur de novo. Molecular diagnosis is made by PCR amplification of STS type sequences (sequence-tagged sites) from the AZFa, b, and c regions. All chromosome Y deletions do not necessarily lead to infertility: firstly, some deletions (especially some partial deletions) do not result in spermatogenesis defects; secondly, among men with severe oligospermia, some can father children without infertility treatment. Finally, when mature spermatozoa are found in the sperm or in the testicles, the infertility problem can be solved with medically assisted procreation techniques. However, there is a risk of transmitting the microdeletion to every male infant. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPartial chromosome Y deletion
Follow this link to review classifications for Partial chromosome Y deletion in Orphanet.

Professional guidelines

PubMed

Rucker GB, Mielnik A, King P, Goldstein M, Schlegel PN
J Urol 1998 Dec;160(6 Pt 1):2068-71. doi: 10.1097/00005392-199812010-00034. PMID: 9817325

Recent clinical studies

Etiology

Alsubaie LM, Alsuwat HS, Almandil NB, AlSulaiman A, AbdulAzeez S, Borgio JF
Mol Biol Rep 2020 Oct;47(10):7623-7632. Epub 2020 Sep 29 doi: 10.1007/s11033-020-05832-6. PMID: 32996047

Therapy

Alsubaie LM, Alsuwat HS, Almandil NB, AlSulaiman A, AbdulAzeez S, Borgio JF
Mol Biol Rep 2020 Oct;47(10):7623-7632. Epub 2020 Sep 29 doi: 10.1007/s11033-020-05832-6. PMID: 32996047

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