Anti-DLD Antibody (CAB13296)

Antibody Name:	Anti-DLD Antibody
Antibody SKU:	CAB13296
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 36-300 of human DLD (NP_000099.2).

Application:	WB IHC IF
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:	Human, Mouse
Positive Samples:	MCF7, BT-474, K-562, 293T, Mouse liver

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 36-300 of human DLD (NP_000099.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	ADQP IDAD VTVI GSGP GGYV AAIK AAQL GFKT VCIE KNET LGGT CLNV GCIP SKAL LNNS HYYH MAHG KDFA SRGI EMSE VRLN LDKM MEQK STAV KALT GGIA HLFK QNKV VHVN GYGK ITGK NQVT ATKA DGGT QVID TKNI LIAT GSEV TPFP GITI DEDT IVSS TGAL SLKK VPEK MVVI GAGV IGVE LGSV WQRL GADV TAVE FLGH VGGV GIDM EISK NFQR ILQK QGFK FKLN TKVT GATK KSDG KIDV SIEA ASGG K
Gene ID:	1738
Uniprot:	P09622
Cellular Location:	Mitochondrion matrix
Calculated MW:	43kDa/49kDa/54kDa
Observed MW:	54kDa

Synonyms:

DLD, DLDD, DLDH, E3, GCSL, LAD, PHE3

Background:

This gene encodes a member of the class-I pyridine nucleotide-disulfide oxidoreductase family. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. In homodimeric form, the encoded protein functions as a dehydrogenase and is found in several multi-enzyme complexes that regulate energy metabolism. However, as a monomer, this protein can function as a protease. Mutations in this gene have been identified in patients with E3-deficient maple syrup urine disease and lipoamide dehydrogenase deficiency. Alternative splicing results in multiple transcript variants.

UniProt Protein Function:	DLD: a multi-functional mitochondrial enzyme. An enzymatic component of the mitochondrial glycine cleavage system, the pyruvate dehydrogenase complex (PDHC), the alpha-ketoglutarate dehydrogenase complex, and the branched-chain alpha-keto acide dehydrogenase complex. Is the E3 component of the PDHC that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2. The E3 component has dihydrolipoamide dehydrogenase activity. The PDHC contains 20-30 copies of pyruvate decarboxylase tetramers (2 alpha:2 beta)(E1), 60 copies of dihydrolipoamide acetyltransferase (E2), six homodimers of dihydrolipoamide dehydrogenase (E3), plus E3 binding proteins. Defects in DLD are a cause of maple syrup urine disease (MSUD), characterized by mental and physical retardation, feeding problems and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine, resulting from a block in oxidative decarboxylation. Differentially expressed in the Wernicke's Area from patients with schizophrenia. Inhibited by 5-methoxyindole-2-carboxylic acid (MICA).
UniProt Protein Details:	Protein type:Amino Acid Metabolism - glycine, serine and threonine; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Oxidoreductase; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Mitochondrial; EC 1.8.1.4; Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - citrate (TCA) cycle Chromosomal Location of Human Ortholog: 7q31-q32 Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; acrosomal matrix; cilium Molecular Function:mercury (II) reductase activity; FAD binding; mercury ion binding; dihydrolipoyl dehydrogenase activity; NADP binding Biological Process: cellular metabolic process; regulation of membrane potential; mitochondrial electron transport, NADH to ubiquinone; cell redox homeostasis; tricarboxylic acid cycle; detoxification of mercury ion; lysine catabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; gastrulation; branched chain family amino acid catabolic process; proteolysis; pyruvate metabolic process; sperm capacitation Disease: Dihydrolipoamide Dehydrogenase Deficiency
NCBI Summary:	This gene encodes a member of the class-I pyridine nucleotide-disulfide oxidoreductase family. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. In homodimeric form, the encoded protein functions as a dehydrogenase and is found in several multi-enzyme complexes that regulate energy metabolism. However, as a monomer, this protein can function as a protease. Mutations in this gene have been identified in patients with E3-deficient maple syrup urine disease and lipoamide dehydrogenase deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014]
UniProt Code:	P09622
NCBI GenInfo Identifier:	269849557
NCBI Gene ID:	1738
NCBI Accession:	P09622.2
UniProt Secondary Accession:	P09622,Q14131, Q14167, Q59EV8, Q8WTS4, B2R5X0, B4DHG0 B4DT69,
UniProt Related Accession:	P09622
Molecular Weight:	509
NCBI Full Name:	Dihydrolipoyl dehydrogenase, mitochondrial
NCBI Synonym Full Names:	dihydrolipoamide dehydrogenase
NCBI Official Symbol:	DLD
NCBI Official Synonym Symbols:	E3; LAD; DLDD; DLDH; GCSL; PHE3
NCBI Protein Information:	dihydrolipoyl dehydrogenase, mitochondrial; diaphorase; lipoamide reductase; lipoamide dehydrogenase; glycine cleavage system L protein; glycine cleavage system protein L; E3 component of pyruvate dehydrogenase complex, 2-oxo-glutarate complex, branched chain keto acid dehydrogenase complex
UniProt Protein Name:	Dihydrolipoyl dehydrogenase, mitochondrial
UniProt Synonym Protein Names:	Dihydrolipoamide dehydrogenase; Glycine cleavage system L protein
Protein Family:	Delta-like protein
UniProt Gene Name:	DLD
UniProt Entry Name:	DLDH_HUMAN

	Western blot analysis of extracts of various cell lines, using DLD antibody (CAB13296) 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: 5s.
	Immunohistochemistry of paraffin-embedded human liver damage using DLD antibody (CAB13296) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded human esophageal cancer using DLD antibody (CAB13296) at dilution of 1:100 (40x lens).
	Immunofluorescence analysis of HeLa cells using DLD antibody (CAB13296). Blue: DAPI for nuclear staining.

Antibodies	ELISA Kits
Anti-DLD Antibody (CAB5403)	Human DLD / Dihydrolipoyl Dehydrogenase ELISA Kit
Anti-DLDH Antibody (CAB5220)	Human DLD (Dihydrolipoyl Dehydrogenase) CLIA Kit (HUES00708)
DLD Antibody (PACO08850)	Human DLD (Dihydrolipoyl Dehydrogenase) ELISA Kit (HUES02307)
	Human Dihydrolipoyl dehydrogenase, mitochondrial (DLD) ELISA Kit

Secondary Antibody

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