Early changes in gene expression in two models of Batten disease.

Abstract:

:Infantile and juvenile neuronal ceroid lipofuscinosis (NCLs) are progressive neurodegenerative disorders of childhood with distinct ages of clinical onset, but with a similar pathological outcome. Infantile and juvenile NCL are inherited in an autosomal recessive manner due to mutations in the CLN1 and CLN3 genes, respectively. Recently developed Cln1- and Cln3-knockout mouse models share similarities in pathology with the respective human disease. Using oligonucleotide arrays we identified reproducible changes in gene expression in the brains of both 10-week-old Cln1- and Cln3-knockout mice as compared to wild-type controls, and confirmed changes in levels of several of the cognate proteins by immunoblotting. Despite the similarities in pathology, the two mutations affect the expression of different, non-overlapping sets of genes. The possible significance of these changes and the pathological mechanisms underlying NCL diseases are discussed.

journal_name

FEBS Lett

journal_title

FEBS letters

authors

Elshatory Y,Brooks AI,Chattopadhyay S,Curran TM,Gupta P,Ramalingam V,Hofmann SL,Pearce DA

doi

10.1016/s0014-5793(03)00162-5

keywords:

subject

Has Abstract

pub_date

2003-03-13 00:00:00

pages

207-12

issue

1-3

eissn

0014-5793

issn

1873-3468

pii

S0014579303001625

journal_volume

538

pub_type

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