Anti-DPM1抗体Dolichol-phosphate mannose （Dol-P-Man） serves as a donor of mannosyl residues on the lumenal side of the endoplasmic reticulum

产品编号 yb-14419R
英文名称 Anti-DPM1抗体
中文名称 DPM1蛋白抗体
别    名 CDGIE; dolichol monophosphate mannose synthase; Dolichol phosphate mannose synthase; Dolichol-phosphate mannose synthase; Dolichol-phosphate mannosyltransferase; Dolichyl phosphate beta D mannosyltransferase; dolichyl phosphate mannosyltransferase polypeptide 1; dolichyl phosphate mannosyltransferase polypeptide 1 catalytic subunit; Dolichyl-phosphate beta-D-mannosyltransferase; DPM synthase; DPM1; DPM1_HUMAN; Mannose P dolichol synthase; Mannose-P-dolichol synthase; MPD synthase; MPDS.
Anti-DPM1抗体 
说 明 书 0.1ml  0.2ml  
研究领域 细胞生物  神经生物学  细胞类型标志物  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, 
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.
分 子 量 30kDa
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human DPM1
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

PubMed PubMed
产品介绍 background:
Dolichol-phosphate mannose (Dol-P-Man) serves as a donor of mannosyl residues on the lumenal side of the endoplasmic reticulum (ER). Lack of Dol-P-Man results in defective surface expression of GPI-anchored proteins. Dol-P-Man is synthesized from GDP-mannose and dolichol-phosphate on the cytosolic side of the ER by the enzyme dolichyl-phosphate mannosyltransferase. Human DPM1 lacks a carboxy-terminal transmembrane domain and signal sequence and is regulated by DPM2. [provided by RefSeq, Jul 2008]

Function:
Transfers mannose from GDP-mannose to dolichol monophosphate to form dolichol phosphate mannose (Dol-P-Man) which is the mannosyl donor in pathways leading to N-glycosylation, glycosyl phosphatidylinositol membrane anchoring, and O-mannosylation of proteins.

Subcellular Location:
Endoplasmic reticulum.

DISEASE:
Defects in DPM1 are the cause of congenital disorder of glycosylation type 1E (CDG1E) [MIM:608799]. CDGs are metabolic deficiencies in glycoprotein biosynthesis that usually cause severe mental and psychomotor retardation. They are characterized by under-glycosylated serum glycoproteins. CDG1E is an autosomal recessive disorder, characterized by severe developmental delay, hypotnia, seizures, and dysmorphic features.

Similarity:
Belongs to the glycosyltransferase 2 family.