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 Lettjournal_title
FEBS lettersauthors
Elshatory Y,Brooks AI,Chattopadhyay S,Curran TM,Gupta P,Ramalingam V,Hofmann SL,Pearce DAdoi
10.1016/s0014-5793(03)00162-5keywords:
subject
Has Abstractpub_date
2003-03-13 00:00:00pages
207-12issue
1-3eissn
0014-5793issn
1873-3468pii
S0014579303001625journal_volume
538pub_type
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