DMGDH Antibody (PACO57532)
- SKU:
- PACO57532
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | DMGDH Antibody (PACO57532) |
抗体コード: | PACO57532 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC, IF |
推奨される希釈: | ELISA:1:2000-1:10000, WB:1:500-1:5000, IHC:1:200-1:500, IF:1:50-1:200 |
反応性: | Human, Mouse |
免疫原: | Recombinant Human Dimethylglycine dehydrogenase, mitochondrial protein (429-524AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
![]() | Western Blot. Positive WB detected in: Mouse liver tissue, Mouse kidney tissue. All lanes: DMGDH antibody at 6.7µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 97, 46 kDa. Observed band size: 97 kDa. |
![]() | IHC image of PACO57532 diluted at 1:300 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
![]() | Immunofluorescence staining of HepG2 cells with PACO57532 at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
バックグラウンド: | Catalyzes the demethylation of N,N-dimethylglycine to sarcosine. Also has activity with sarcosine in vitro. |
シノニム: | Dimethylglycine dehydrogenase, mitochondrial (EC 1.5.8.4) (ME2GLYDH), DMGDH |
UniProt Protein Function: | DMGDH: Defects in DMGDH are the cause of DMGDH deficiency (DMGDHD). DMGDHD is a disorder characterized by fish odor, muscle fatigue with increased serum creatine kinase. Biochemically it is characterized by an increase of N,N- dimethylglycine (DMG) in serum and urine. Belongs to the GcvT family. |
UniProt Protein Details: | Protein type:Amino Acid Metabolism - glycine, serine and threonine; EC 1.5.8.4; Mitochondrial; Oxidoreductase Chromosomal Location of Human Ortholog: 5q14.1 Cellular Component: mitochondrial matrix Molecular Function:dimethylglycine dehydrogenase activity; electron carrier activity Biological Process: betaine catabolic process; choline metabolic process; glycine metabolic process Disease: Dimethylglycine Dehydrogenase Deficiency |
NCBI Summary: | This gene encodes an enzyme involved in the catabolism of choline, catalyzing the oxidative demethylation of dimethylglycine to form sarcosine. The enzyme is found as a monomer in the mitochondrial matrix, and uses flavin adenine dinucleotide and folate as cofactors. Mutation in this gene causes dimethylglycine dehydrogenase deficiency, characterized by a fishlike body odor, chronic muscle fatigue, and elevated levels of the muscle form of creatine kinase in serum. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013] |
UniProt Code: | Q9UI17 |
NCBI GenInfo Identifier: | 296434575 |
NCBI Gene ID: | 29958 |
NCBI Accession: | Q9UI17.2 |
UniProt Secondary Accession: | Q9UI17,B2RBN0, B4E1J9, |
UniProt Related Accession: | Q9UI17 |
Molecular Weight: | 45,079 Da |
NCBI Full Name: | Dimethylglycine dehydrogenase, mitochondrial |
NCBI Synonym Full Names: | dimethylglycine dehydrogenase |
NCBI Official Symbol: | DMGDH |
NCBI Official Synonym Symbols: | DMGDHD; ME2GLYDH |
NCBI Protein Information: | dimethylglycine dehydrogenase, mitochondrial |
UniProt Protein Name: | Dimethylglycine dehydrogenase, mitochondrial |
UniProt Synonym Protein Names: | ME2GLYDH |
Protein Family: | Dimethylglycine dehydrogenase |
UniProt Gene Name: | DMGDH |
UniProt Entry Name: | M2GD_HUMAN |
Antibodies | ELISA Kits |
DMGDH Antibody (PACO00712) | Human DMGDH / Dimethylglycine dehydrogenase ELISA Kit |
Human Dimethylglycine dehydrogenase, mitochondrial (DMGDH) ELISA Kit |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
Recommended Products |
Anti-FITC Goat Anti-Rabbit IgG (H+L) Antibody (CABS011) |
Anti-HRP-conjugated Beta Actin Antibody (CABC028) |