RGD Reference Report - A rat model of human FENIB (familial encephalopathy with neuroserpin inclusion bodies). - Rat Genome Database

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A rat model of human FENIB (familial encephalopathy with neuroserpin inclusion bodies).

Authors: Takano, K  Kitao, Y  Inagi, R  Momoi, T  Matsuyama, T  Miyata, T  Yoneda, Y  Iso, H  Stern, DM  Hori, O  Ogawa, S 
Citation: Takano K, etal., Biochem Biophys Res Commun. 2006 Aug 4;346(3):1040-7. Epub 2006 Jun 12.
RGD ID: 7207386
Pubmed: PMID:16782060   (View Abstract at PubMed)
DOI: DOI:10.1016/j.bbrc.2006.06.016   (Journal Full-text)

FENIB (familial encephalopathy with neuroserpin inclusion bodies) is caused by intracellular accumulation/polymerization of mutant neuroserpins in the endoplasmic reticulum (ER). Transgenic rats overexpressing megsin (Tg meg), a newly identified serine protease inhibitor (serpin), demonstrated intraneuronal periodic-acid Schiff (PAS)-positive inclusions distributed throughout deeper layers of cerebral cortex, CA1 of the hippocampus, and substantia nigra. Hippocampal extracts from Tg meg rats showed increased expression of ER stress proteins, and activation of caspases-12 and -3, associated with decreased neuronal density. Enhanced ER stress was also observed in dopaminergic neurons in the substantia nigra, in parallel with decreased neuronal viability and motor coordination. In each case, PAS-positive inclusions were also positive for megsin. These data suggest that overexpression of megsin results in ER stress, eventuating in the formation of PAS-positive inclusions. Tg meg rats provide a novel model of FENIB, where accumulation of serpins in the ER induces selective dysfunction/loss of specific neuronal populations.

RGD Manual Disease Annotations    Click to see Annotation Detail View

Objects Annotated

Genes (Rattus norvegicus)
Serpinb7  (serpin family B member 7)

Genes (Mus musculus)
Serpinb7  (serine (or cysteine) peptidase inhibitor, clade B, member 7)

Genes (Homo sapiens)
SERPINB7  (serpin family B member 7)


Additional Information