PREPL: a putative novel oligopeptidase propelled into the limelight
PREPL: a putative novel oligopeptidase propelled into the limelight
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DOI：
10.1515/bc.2006.111
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发表时间：
2006-07-01
影响因子：
3.7
通讯作者：
Creemers, John W. M.
中科院分区：
文献类型：
--
作者：
Martens, Kevin;Derua, Rita;Creemers, John W. M.
脯氨酰内肽酶样蛋白PREPL最近引起了人们的注意，因为它的基因位于两个连续的基因缺失综合征，2 p21缺失综合征和低张力-胱氨酸尿综合征。该基因的缺失会导致出生时张力减退、发育不良和生长激素缺乏。PREPL对基于活性的探针具有高度反应性，这表明存在完整的催化机制。然而，还没有找到底物。催化结构域独特的羧基末端可能包含至今难以捉摸的特异性的关键。
The prolyl endopeptidase-like protein PREPL has recently attracted attention because its gene is located within two contiguous gene-deletion syndromes, the 2p21 deletion syndrome and the hypotonia-cystinuria syndrome. Deletion of the gene results in hypotonia at birth, failure to thrive and growth hormone deficiency. PREPL is highly reactive against an activity-based probe, which indicates the presence of an intact catalytic machinery. However, no substrate has been found yet. The unique carboxyterminus of the catalytic domain might contain the key to the as yet elusive specificity.