A lamin A/C beta-strand containing the site of lipodystrophy mutations is a major surface epitope for a new panel of monoclonal antibodies

被引：20

作者：

Manilal, S ^{[1
]}

Randles, KN ^{[1
]}

Aunac, C ^{[1
]}

Man, NT ^{[1
]}

Morris, GE ^{[1
]}

机构：

[1] NE Wales Inst, MRIC Biochem Grp, Wrexham LL11 2AW, Wales

来源：

BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS | 2004年 / 1671卷 / 1-3期

关键词：

phage display; peptide library; epitope mapping; nuclear lamina; Emery-Dreifuss muscular dystrophy;

D O I：

10.1016/j.bbagen.2004.01.008

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Using a phage-displayed peptide library, we have identified the epitope recognized by a new panel of five monoclonal antibodies (mAbs) raised against full-length recombinant human lamin A. The mAbs were found to recognize both lamin A and C by Western blotting and immunolocalization at the nuclear rim. A nine-amino acid consensus sequence PLLTYRFPP in the common immumoglobulin-like (Ig-like) domain of lamin A/C contains the binding site for all five mAbs. Three-dimensional structure of the Ig-like domain of lamin A/C shows this sequence is a complete beta-strand. This sequence includes arginine-482 (R482) which is mutated in most cases of Dunnigan-type familial partial lipodystrophy (FPLD). R482 may be part of an interaction site on the surface of lamin A/C for lamin-binding proteins associated with lipodystrophy. (C) 2004 Elsevier B.V All rights reserved.

引用

页码：87 / 92

页数：6

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