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DPM1 Rabbit pAb (bs-14419R)  
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50ul/1180.00元
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產品編號 bs-14419R
英文名稱 DPM1 Rabbit pAb
中文名稱 DPM1蛋白抗體
別    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 polypep  
研究領域 細胞生物  神經生物學  細胞類型標志物  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human (predicted: Mouse,Rat,Pig,Sheep,Cow,Dog,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 30 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DPM1: 51-150/260 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

SWISS:
O60762

Gene ID:
8813

Database links:

Entrez Gene: 8813 Human

Entrez Gene: 534097 Cow

Entrez Gene: 13480 Mouse

Entrez Gene: 100124379 Pig

Entrez Gene: 296394 Rat

Omim: 603503 Human

SwissProt: Q1JQ93 Cow

SwissProt: O60762 Human

SwissProt: O70152 Mouse

SwissProt: A5GFZ5 Pig

Unigene: 654951 Human

Unigene: 422657 Mouse



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