Anti-D2HGDH 抗体 (CAB16213)

抗体名:	Anti-D2HGDH Antibody
抗体コード:	CAB16213
抗体サイズ:	20uL, 50uL, 100uL
申し込み:	WB
反応性:	Mouse, Rat
宿主種:	Rabbit
免疫原:	Recombinant fusion protein containing a sequence corresponding to amino acids 322-521 of human D2HGDH (NP_689996.4).

申し込み:	WB
推奨希釈:	WB 1:500 - 1:2000
反応性:	Mouse, Rat
ポジティブサンプル:	Mouse liver, Rat heart

免疫原:	Recombinant fusion protein containing a sequence corresponding to amino acids 322-521 of human D2HGDH (NP_689996.4).
精製方法:	Affinity purification
ストレージバッファ:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
アイソタイプ:	IgG
順序:	GRHL HLAS PVQE SPFY VLIE TSGS NAGH DAEK LGHF LEHA LGSG LVTD GTMA TDQR KVKM LWAL RERI TEAL SRDG YVYK YDLS LPVE RLYD IVTD LRAR LGPH AKHV VGYG HLGD GNLH LNVT AEAF SPSL LAAL EPHV YEWT AGQQ GSVS AEHG VGFR KRDV LGYS KPPG ALQL MQQL KALL DPKG ILNP YKTL PSQA
遺伝子ID:	728294
Uniprot:	Q8N465
セルラーロケーション:
計算された分子量:	32kDa/34kDa/56kDa
観察された分子量:	56kDa

同義語:

D2HGDH, D2HGD

バックグラウンド:

This gene encodes D-2hydroxyglutarate dehydrogenase, a mitochondrial enzyme belonging to the FAD-binding oxidoreductase/transferase type 4 family. This enzyme, which is most active in liver and kidney but also active in heart and brain, converts D-2-hydroxyglutarate to 2-ketoglutarate. Mutations in this gene are present in D-2-hydroxyglutaric aciduria, a rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features.

UniProt Protein Function:	D2HGDH: an oxidoreductase that act on the CH-OH group of donor with other acceptors. Catalyzes the oxidation of D-2-hydroxyglutarate (2HG) to alpha-ketoglutarate. Belongs to the FAD-binding oxidoreductase/transferase type 4 family. 2 human isoforms produced by alternative splicing. Defects in D2HGDH are the cause of D-2-hydroxyglutaric aciduria (D2HGA), a rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. Both a mild and a severe phenotype exist. The severe phenotype is homogeneous and is characterized by early infantile-onset epileptic encephalopathy and cardiomyopathy. The mild phenotype has a more variable clinical presentation. Diagnosis is based on the presence of an excess of D-2-hydroxyglutaric acid in the urine.
UniProt Protein Details:	Protein type:EC 1.1.99.-; Mitochondrial; Oxidoreductase Chromosomal Location of Human Ortholog: 2q37.3 Cellular Component: mitochondrial matrix; mitochondrion Molecular Function:(R)-2-hydroxyglutarate dehydrogenase activity; D-lactate dehydrogenase (cytochrome) activity Biological Process: 2-oxoglutarate metabolic process; cellular protein metabolic process; lactate oxidation; response to calcium ion; response to cobalt ion; response to magnesium ion; response to manganese ion; response to zinc ion Disease: D-2-hydroxyglutaric Aciduria 1
NCBI Summary:	This gene encodes D-2hydroxyglutarate dehydrogenase, a mitochondrial enzyme belonging to the FAD-binding oxidoreductase/transferase type 4 family. This enzyme, which is most active in liver and kidney but also active in heart and brain, converts D-2-hydroxyglutarate to 2-ketoglutarate. Mutations in this gene are present in D-2-hydroxyglutaric aciduria, a rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. [provided by RefSeq, Jul 2008]
UniProt Code:	Q8N465
NCBI GenInfo Identifier:	564730541
NCBI Gene ID:	728294
NCBI Accession:	NP_001274178.1
UniProt Secondary Accession:	Q8N465,Q6IQ24, Q8N5Q8, B4E3L6, E7ENP2,
UniProt Related Accession:	Q8N465
Molecular Weight:	70.83kD
NCBI Full Name:	D-2-hydroxyglutarate dehydrogenase, mitochondrial isoform 2
NCBI Synonym Full Names:	D-2-hydroxyglutarate dehydrogenase
NCBI Official Symbol:	D2HGDH
NCBI Official Synonym Symbols:	D2HGD
NCBI Protein Information:	D-2-hydroxyglutarate dehydrogenase, mitochondrial
UniProt Protein Name:	D-2-hydroxyglutarate dehydrogenase, mitochondrial
Protein Family:	D-2-hydroxyglutarate dehydrogenase
UniProt Gene Name:	D2HGDH

Western blot analysis of extracts of various cell lines, using D2HGDH antibody (CAB16213) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.

Williams et al.

Signalling metabolite L-2-hydroxyglutarate activates the transcription factor HIF-1Î± in lipopolysaccharide-activated macrophages

J Biol Chem.2021

PubMed ID: 34929172

Antibodies

D2HGDH Antibody (PACO08731)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)