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Testicular degeneration in Huntington disease.

Abstract：

:Huntington disease (HD) is an adult onset, neurodegenerative disorder that results from CAG expansion in the HD gene. Recent work has demonstrated testicular degeneration in mouse models of HD and alterations in the hypothalamic-pituitary-gonadal (HPG) axis in HD patients. Here, we show that HD patients have specific testicular pathology with reduced numbers of germ cells and abnormal seminiferous tubule morphology. In the YAC128 mouse model, testicular degeneration develops prior to 12 months of age, but at 12 months, there is no evidence for decreased testosterone levels or loss of GnRH neurons in the hypothalamus. This suggests that testicular pathology results from a direct toxic effect of mutant huntingtin in the testis and is supported by the fact that huntingtin is highly expressed in the affected cell populations in the testis. Understanding the pathogenesis of HD in the testis may reveal common critical pathways which lead to degeneration in both the brain and testis.

journal_name

Neurobiol Dis

journal_title

Neurobiology of disease

authors

Van Raamsdonk JM,Murphy Z,Selva DM,Hamidizadeh R,Pearson J,Petersén A,Björkqvist M,Muir C,Mackenzie IR,Hammond GL,Vogl AW,Hayden MR,Leavitt BR

doi

10.1016/j.nbd.2007.01.006

subject

Has Abstract

pub_date

2007-06-01 00:00:00

pages

512-20

issue

3

eissn

0969-9961

issn

1095-953X

pii

S0969-9961(07)00022-8

journal_volume

26

pub_type

杂志文章

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