Presence of BACE1 and BACE2 in muscle fibres of patients with sporadic inclusion-body myositis

Gaetano Vattemi; W. King Engel; Janis McFerrin; Joseph D. Buxbaum; Lucia Pastorino; Valerie Askanas

doi:10.1016/S0140-6736(01)06969-0

Presence of BACE1 and BACE2 in muscle fibres of patients with sporadic inclusion-body myositis

Gaetano Vattemi, W. King Engel, Janis McFerrin, Joseph D. Buxbaum, Lucia Pastorino, Valerie Askanas

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Abstract

Sporadic inclusion-body myositis (IBM) is the most common, progressive muscle disease of older individuals. We investigated the presence of BACE1 and BACE2 - two β secretases that cleave amyloid-β-precursor protein - in muscle-biopsy samples from patients with IBM and from controls. On immunofluorescence, BACE1 and BACE2 co-localised with amyloid β in IBM vacuolated muscle fibres, but were not found in controls. Immunoblotting showed increased BACE2 but not BACE1 in patients with IBM compared with controls. Our study suggests that both of these proteases might participate in processing of amyloid-β-precursor protein in IBM muscle fibres.

Original language	English
Pages (from-to)	1962-1964
Number of pages	3
Journal	The Lancet
Volume	358
Issue number	9297
DOIs	https://doi.org/10.1016/S0140-6736(01)06969-0
State	Published - 8 Dec 2001

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title = "Presence of BACE1 and BACE2 in muscle fibres of patients with sporadic inclusion-body myositis",

abstract = "Sporadic inclusion-body myositis (IBM) is the most common, progressive muscle disease of older individuals. We investigated the presence of BACE1 and BACE2 - two β secretases that cleave amyloid-β-precursor protein - in muscle-biopsy samples from patients with IBM and from controls. On immunofluorescence, BACE1 and BACE2 co-localised with amyloid β in IBM vacuolated muscle fibres, but were not found in controls. Immunoblotting showed increased BACE2 but not BACE1 in patients with IBM compared with controls. Our study suggests that both of these proteases might participate in processing of amyloid-β-precursor protein in IBM muscle fibres.",

author = "Gaetano Vattemi and Engel, {W. King} and Janis McFerrin and Buxbaum, {Joseph D.} and Lucia Pastorino and Valerie Askanas",

note = "Funding Information: This work was supported by grants from the National Institutes of Health (AG16768 [to VA] and AG4996 [to BDB]), the Muscular Dystrophy Association (to VA), and the Helen Lewis Research Fund.",

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AU - McFerrin, Janis

AU - Buxbaum, Joseph D.

AU - Pastorino, Lucia

AU - Askanas, Valerie

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N2 - Sporadic inclusion-body myositis (IBM) is the most common, progressive muscle disease of older individuals. We investigated the presence of BACE1 and BACE2 - two β secretases that cleave amyloid-β-precursor protein - in muscle-biopsy samples from patients with IBM and from controls. On immunofluorescence, BACE1 and BACE2 co-localised with amyloid β in IBM vacuolated muscle fibres, but were not found in controls. Immunoblotting showed increased BACE2 but not BACE1 in patients with IBM compared with controls. Our study suggests that both of these proteases might participate in processing of amyloid-β-precursor protein in IBM muscle fibres.

AB - Sporadic inclusion-body myositis (IBM) is the most common, progressive muscle disease of older individuals. We investigated the presence of BACE1 and BACE2 - two β secretases that cleave amyloid-β-precursor protein - in muscle-biopsy samples from patients with IBM and from controls. On immunofluorescence, BACE1 and BACE2 co-localised with amyloid β in IBM vacuolated muscle fibres, but were not found in controls. Immunoblotting showed increased BACE2 but not BACE1 in patients with IBM compared with controls. Our study suggests that both of these proteases might participate in processing of amyloid-β-precursor protein in IBM muscle fibres.

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Presence of BACE1 and BACE2 in muscle fibres of patients with sporadic inclusion-body myositis

Abstract

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