antibody

DPAGT1 Antibody

MBS150075

0.1 mg

345 USD

polyclonal

rabbit

nonconjugated

human, mouse

western blot, ELISA, immunohistochemistry, immunocytochemistry, enzyme immunoassay

Antibody

DPAGT1 Antibody

DPAGT1

DPAGT1; GPT; ALG7; DGPT; G1PT; UAGT; UGAT; CDG1J; DPAGT; CDG-Ij; CMSTA2; DPAGT2; D11S366; UDP-N-acetylglucosamine--dolichyl-phosphate N-acetylglucosaminephosphotransferase; GlcNAc-1-P transferase; dolichyl-phosphate (UDP-N-acetylglucosamine) N-acetylglucosaminephosphotransferase 1 (GlcNAc-1-P transferase)

UDP-N-acetylglucosamine--dolichyl-phosphate N-acetylglucosaminephosphotransferase; UDP-N-acetylglucosamine--dolichyl-phosphate N-acetylglucosaminephosphotransferase; UDP-N-acetylglucosamine--dolichyl-phosphate N-acetylglucosaminephosphotransferase; GlcNAc-1-P transferase; N-acetylglucosamine-1-phosphate transferase; dolichyl-phosphate alpha-N-acetylglucosaminyltransferase; UDP-GlcNAc:dolichyl-phosphate N-acetylglucosaminephosphotransferase; dolichyl-phosphate (UDP-N-acetylglucosamine) N-acetylglucosaminephosphotransferase 1 (GlcNAc-1-P tra; dolichyl-phosphate (UDP-N-acetylglucosamine) N-acetylglucosaminephosphotransferase 1 (GlcNAc-1-P transferase); GlcNAc-1-P transferase; G1PT; GPT; N-acetylglucosamine-1-phosphate transferase

DPAGT1

DPAGT1; DPAGT1; GPT; ALG7; DGPT; G1PT; UAGT; UGAT; CDG1J; DPAGT; CDG-Ij; CMSTA2; DPAGT2; D11S366; DPAGT2; G1PT; GPT

GPT_HUMAN

Polyclonal

IgG

Rabbit

Human, Mouse

408

At least four isoforms of DPAGT1 are known to exist; this antibody will recognize the two longest isoforms. DPAGT1 antibody is predicted to not cross-react with UHRF1BP1.

DPAGT1 Antibody is affinity chromatography purified via peptide column.

Liquid

1 mg/mL

DPAGT1 antibody can be stored at 4 degree C for three months and -20 degree C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

DPAGT1 antibody can be used for detection of DPAGT1 by Western blot at 1 mug/mL. Antibody can also be used for immunohistochemistry starting at 2.5 mug/mL. For immunofluorescence start at 20 mug/mL.

Conjugate: Unconjugated. Immunogen: DPAGT1 antibody was raised against a 17 amino acid synthetic peptide near the amino terminus of human DPAGT1. Buffer: DPAGT1 Antibody is supplied in PBS containing 0.02% sodium azide.

DPAGT1 Antibody: The UDP-N-acetylglucosamine-dolichyl-phosphate N-acetyl-glucosaminephosphotransferase (DPAGT1) is an enzyme that catalyzes the first step in the dolichol-linked oligosaccharide pathway for glycoprotein biosynthesis. Mutations in this integral endoplasmic reticulum (ER) membrane protein enzyme belongs to the glycosyltransferase family 4 results in the congenital disorder of glycosylation type Ij with symptoms such as severe hypotonia, medically intractable seizures, mental retardation, microcephaly, and exotropia. Recent experiments have shown that DPAGT1 is a target of the Wnt/beta-catenin signaling pathway, with Wnt3a inducing higher DPAGT1 mRNA expression.

42794009

NP_001373

NM_001382.3

Q9H3H5

34,270 Da

Asparagine N-linked Glycosylation Pathway (161013); Biosynthesis Of The N-glycan Precursor (dolichol Lipid-linked Oligosaccharide, LLO) And Transfer To A Nascent Protein Pathway (161014); Defective ALG1 Causes ALG1-CDG (CDG-1k) Pathway (1127605); Defective ALG11 Causes ALG11-CDG (CDG-1p) Pathway (1127608); Defective ALG12 Causes ALG12-CDG (CDG-1g) Pathway (1127601); Defective ALG14 Causes Congenital Myasthenic Syndrome (ALG14-CMS) Pathway (1127613); Defective ALG2 Causes ALG2-CDG (CDG-1i) Pathway (1127603); Defective ALG3 Causes ALG3-CDG (CDG-1d) Pathway (1127599); Defective ALG6 Causes ALG6-CDG (CDG-1c) Pathway (1127597); Defective ALG8 Causes ALG8-CDG (CDG-1h) Pathway (1127602)

The protein encoded by this gene is an enzyme that catalyzes the first step in the dolichol-linked oligosaccharide pathway for glycoprotein biosynthesis. This enzyme belongs to the glycosyltransferase family 4. This protein is an integral membrane protein of the endoplasmic reticulum. The congenital disorder of glycosylation type Ij is caused by mutation in the gene encoding this enzyme. [provided by RefSeq, Jul 2008]

DPAGT1: Catalyzes the initial step in the synthesis of dolichol- P-P-oligosaccharides. Defects in DPAGT1 are the cause of congenital disorder of glycosylation type 1J (CDG1J). 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. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. Belongs to the glycosyltransferase 4 family. 3 isoforms of the human protein are produced by alternative splicing. Protein type: Transferase; EC 2.7.8.15; Membrane protein, integral; Membrane protein, multi-pass; Glycan Metabolism - N-glycan biosynthesis; Endoplasmic reticulum. Chromosomal Location of Human Ortholog: 11q23.3. Cellular Component: endoplasmic reticulum membrane; intracellular membrane-bound organelle; membrane; integral to membrane; integral to endoplasmic reticulum membrane. Molecular Function: UDP-N-acetylglucosamine-dolichyl-phosphate N-acetylglucosaminephosphotransferase activity; transferase activity, transferring glycosyl groups; phospho-N-acetylmuramoyl-pentapeptide-transferase activity. Biological Process: polysaccharide biosynthetic process; UDP-N-acetylglucosamine metabolic process; cellular protein metabolic process; dolichol-linked oligosaccharide biosynthetic process; protein amino acid N-linked glycosylation; protein amino acid N-linked glycosylation via asparagine; dolichol biosynthetic process; post-translational protein modification; protein oligomerization. Disease: Congenital Disorder Of Glycosylation, Type Ij; Myasthenic Syndrome, Congenital, With Tubular Aggregates 2

0.1 mg

345 USD