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PDLIM3
Actin-associated LIM protein (ALP), also known as PDZ and LIM domain protein 3 is a protein that in humans is encoded by the ''PDLIM3'' gene.〔(【引用サイトリンク】 url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=27295 )〕 ALP is highly expressed in cardiac and skeletal muscle, where it localizes to Z-discs and intercalated discs. ALP functions to enhance the crosslinking of actin by alpha actinin-2 and also appears to be essential for right ventricular chamber formation and contractile function.
== Structure ==
ALP exists primarily as two alternatively spliced variants; a 39.2 kDa (364 amino acids) protein in skeletal muscle and a 34.3 kDa (316 amino acids) protein in cardiac muscle and smooth muscle.〔(【引用サイトリンク】url=http://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=Q53GG5-2 )〕〔(【引用サイトリンク】 url = http://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=Q53GG5 )〕 ALP has a N-terminal PDZ domain and a C-terminal LIM domain. In addition, the ALP subfamily contains a specific 34 amino acid domain named the ALP-like motif, containing protein kinase C consensus sequences. The PDZ domain of ALP binds to alpha actinin-2, specifically to its spectrin-like repeats. The PDZ domain is a motif composed of 80-120 amino acids with conserved four residue GLGF sequences that typically interact with C-termini of cytoskeletal proteins. The region of heterogeneity in the two isoforms is between the PDZ domain and LIM domain.〔 ALP is localized to chromosome 4q35.〔 It has been shown that deletion of muscleblind-like 1 in mice can alter the splicing pattern of ''PDLIM3''.
抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』
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