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Rabbit Anti-DPM1/BF594 Conjugated antibody (bs-14419R-BF594)
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說(shuō) 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-14419R-BF594
英文名稱 Rabbit Anti-DPM1/BF594 Conjugated antibody
中文名稱 BF594標(biāo)記的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 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.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說(shuō) 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  神經(jīng)生物學(xué)  細(xì)胞類型標(biāo)志物  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng)
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 30kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human DPM1
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產(chǎn)品介紹 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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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