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Product Name Anti-DPM1/AF594 Chinese Name AF594标记的DPM1蛋白抗体 Alias 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. Research Area Cell biology Neurobiology Cell type markers Immunogen Species Rabbit Clonality Polyclonal React Species Applications ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 30kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human DPM1 Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage 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. Product Detail 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: 13480 Mouse
Omim: 603503 Human
SwissProt: O60762 Human
SwissProt: O70152 Mouse
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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