MPDU1蛋白抗体 MPDU1

MPDU1蛋白抗体

规格：1mg/1ml

英文名： MPDU1

别名： CDGIF; LEC35; mannose P dolichol utilization defect 1; Mannose-P-dolichol utilization defect 1 protein; MPDU1; MPU1_HUMAN; PQLC5; SL15; Suppressor of Lec15 and Lec35 glycosylation mutation homolog.

分子量： 26kDa

储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆类型：Polyclonal

亚型：IgG

纯化方法：affinity purified by Protein A

**原：KLH conjugated synthetic peptide derived from human MPDU1

交叉反应：Human, Mouse, Rat, Dog, Cow, Horse, Sheep,

细胞定位：细胞膜

MPDU1蛋白抗体产品介绍：background: This gene encodes an endoplasmic reticulum membrane protein that is required for utilization of the mannose donor mannose-P-dolichol in the synthesis of lipid-linked oligosaccharides and glycosylphosphatidylinositols. Mutations in this gene result in congenital disorder of glycosylation type If. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2008] Function: Required for normal utilization of mannose-dolichol phosphate (Dol-P-Man) in the synthesis of N-linked and O-linked oligosaccharides and GPI anchors. Subcellular Location: Membrane. DISEASE: Defects in MPDU1 are the cause of congenital disorder of glycosylation type MPDU1蛋白抗体1F (CDG1F) [MIM:609180]. CDGs are a family of severe inherited diseases caused by a defect in protein N-glycosylation. They are characterized by under-glycosylated serum proteins. These multisystem disorders present with a wide variety of clinical features, such as disorders of the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency.MPDU1蛋白抗体 The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. Similarity: Belongs to the MPDU1 (TC 2.A.43.3) family. Contains 2 PQ-loop domains. Gene ID: 9526 Database links: Entrez Gene: 9526 Human Omim: 604041 Human SwissProt: O75352 Human Unigene: 246381 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

MPDU1蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.

研究领域：肿瘤  细胞生物  信号转导  

储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源： Rabbit

外观： Lyophilized or Liquid