ALG1 Antibody (PACO07695)
- SKU:
- PACO07695
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | ALG1 Antibody (PACO07695) |
抗体コード: | PACO07695 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC |
推奨される希釈: | |
反応性: | Human, Mouse, Rat |
免疫原: | Human ALG1 |
憲法: | Liquid |
ストレージバッファ: | PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
精製方法: | Antigen Affinity Purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
シノニム: | asparagine-linked glycosylation 1, β-1,4-mannosyltransferase homolog (S. cerevisiae);ALG1;HMAT1;HMT-1;HMT1 ; |
UniProt Protein Function: | ALG1: Participates in the formation of the lipid-linked precursor oligosaccharide for N-glycosylation. Involved in assembling the dolichol-pyrophosphate-GlcNAc(2)-Man(5) intermediate on the cytoplasmic surface of the ER. Defects in ALG1 are the cause of congenital disorder of glycosylation type 1K (CDG1K). 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 group 1 family. Glycosyltransferase 33 subfamily. |
UniProt Protein Details: | Protein type:Endoplasmic reticulum; Glycan Metabolism - N-glycan biosynthesis; EC 2.4.1.142; Membrane protein, integral; Transferase Chromosomal Location of Human Ortholog: 16p13.3 Cellular Component: endoplasmic reticulum membrane; membrane; endoplasmic reticulum; integral to membrane Molecular Function:mannosyltransferase activity; chitobiosyldiphosphodolichol beta-mannosyltransferase activity Biological Process: cellular protein metabolic process; dolichol-linked oligosaccharide biosynthetic process; lipopolysaccharide biosynthetic process; protein amino acid glycosylation; protein amino acid N-linked glycosylation via asparagine; post-translational protein modification Disease: Congenital Disorder Of Glycosylation, Type Ik |
NCBI Summary: | The enzyme encoded by this gene catalyzes the first mannosylation step in the biosynthesis of lipid-linked oligosaccharides. This gene is mutated in congenital disorder of glycosylation type Ik. [provided by RefSeq, Dec 2008] |
UniProt Code: | Q9BT22 |
NCBI GenInfo Identifier: | 73921663 |
NCBI Gene ID: | 56052 |
NCBI Accession: | Q9BT22.2 |
UniProt Related Accession: | Q9BT22 |
Molecular Weight: | Observed: 53 kDaPredicted: 53 kDa |
NCBI Full Name: | Chitobiosyldiphosphodolichol beta-mannosyltransferase |
NCBI Synonym Full Names: | ALG1 chitobiosyldiphosphodolichol beta-mannosyltransferase |
NCBI Official Symbol: | ALG1 |
NCBI Official Synonym Symbols: | HMT1; MT-1; CDG1K; HMAT1; HMT-1; Mat-1; hMat-1 |
NCBI Protein Information: | chitobiosyldiphosphodolichol beta-mannosyltransferase |
UniProt Protein Name: | Chitobiosyldiphosphodolichol beta-mannosyltransferase |
UniProt Synonym Protein Names: | Asparagine-linked glycosylation protein 1 homolog; Beta-1,4-mannosyltransferase; GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase; GDP-mannose-dolichol diphosphochitobiose mannosyltransferase; Mannosyltransferase-1; MT-1; hMat-1 |
UniProt Gene Name: | ALG1 |
UniProt Entry Name: | ALG1_HUMAN |