Cell Biology Antibodies 6

Anti-SDHD Antibody (CAB16240)

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SKU:
CAB16240
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Cell Biology

Description

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Antibody Name:Anti-SDHD Antibody
Antibody SKU:CAB16240
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse, Rat
Host Species:Rabbit
Immunogen:A synthetic peptide corresponding to a sequence within amino acids 50-150 of human SDHD (NP_002993.1).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse, Rat
Positive Samples:mouse brain, mouse heart, mouse kidney
Immunogen:A synthetic peptide corresponding to a sequence within amino acids 50-150 of human SDHD (NP_002993.1).
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:HLSP SHHS GSKA ASLH WTSE RVVS VLLL GLLP AAYL NPCS AMDY SLAA ALTL HGHW GLGQ VVTD YVHG DALQ KAAK AGLL ALSA LTFA GLCY FNYH DVGI C
Gene ID:6392
Uniprot:O14521
Cellular Location:Mitochondrion inner membrane, Multi-pass membrane protein
Calculated MW:9kDa/12kDa/15kDa/17kDa
Observed MW:17kDa
Synonyms:SDHD, CBT1, CII-4, CWS3, PGL, PGL1, QPs3, SDH4, cybS
Background:This gene encodes a member of complex II of the respiratory chain, which is responsible for the oxidation of succinate. The encoded protein is one of two integral membrane proteins anchoring the complex to the matrix side of the mitochondrial inner membrane. Mutations in this gene are associated with the formation of tumors, including hereditary paraganglioma. Transmission of disease occurs almost exclusively through the paternal allele, suggesting that this locus may be maternally imprinted. There are pseudogenes for this gene on chromosomes 1, 2, 3, 7, and 18. Alternative splicing results in multiple transcript variants.
UniProt Protein Function:SDHD: Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Defects in SDHD are a cause of paragangliomas type 1 (PGL1). A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion. PGL1 is a rare autosomal dominant disorder which is characterized by the development of mostly benign, highly vascular, slowly growing tumors in the head and neck. In the head and neck region, the carotid body is the largest of all paraganglia and is also the most common site of the tumors. Defects in SDHD are a cause of susceptibility to pheochromocytoma (PCC). A catecholamine-producing tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent. Defects in SDHD may be a cause of susceptibility to intestinal carcinoid tumor (ICT). A yellow, well- differentiated, circumscribed tumor that arises from enterochromaffin cells in the small intestine or, less frequently, in other parts of the gastrointestinal tract. Defects in SDHD are a cause of paraganglioma and gastric stromal sarcoma (PGGSS); also called Carney-Stratakis syndrome. Gastrointestinal stromal tumors may be sporadic or inherited in an autosomal dominant manner, alone or as a component of a syndrome associated with other tumors, such as in the context of neurofibromatosis type 1 (NF1). Patients have both gastrointestinal stromal tumors and paragangliomas. Susceptibility to the tumors was inherited in an apparently autosomal dominant manner, with incomplete penetrance. Defects in SDHD are a cause of Cowden-like syndrome (CWDLS). Cowden-like syndrome is a cancer predisposition syndrome associated with elevated risk for tumors of the breast, thyroid, kidney and uterus. Belongs to the CybS family.
UniProt Protein Details:

Protein type:Mitochondrial; Carbohydrate Metabolism - citrate (TCA) cycle; Energy Metabolism - oxidative phosphorylation; Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 11q23

Cellular Component: mitochondrial envelope; mitochondrial inner membrane; mitochondrial respiratory chain complex II; mitochondrion

Molecular Function:electron carrier activity; heme binding; succinate dehydrogenase activity; ubiquinone binding

Biological Process: tricarboxylic acid cycle

Disease: Carcinoid Tumors, Intestinal; Cowden Syndrome 3; Paraganglioma And Gastric Stromal Sarcoma; Paragangliomas 1; Pheochromocytoma

NCBI Summary:This gene encodes a member of complex II of the respiratory chain, which is responsible for the oxidation of succinate. The encoded protein is one of two integral membrane proteins anchoring the complex to the matrix side of the mitochondrial inner membrane. Mutations in this gene are associated with the formation of tumors, including hereditary paraganglioma. Transmission of disease occurs almost exclusively through the paternal allele, suggesting that this locus may be maternally imprinted. There are pseudogenes for this gene on chromosomes 1, 2, 3, 7, and 18. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Feb 2013]
UniProt Code:O14521
NCBI GenInfo Identifier:3913480
NCBI Gene ID:6392
NCBI Accession:O14521.1
UniProt Secondary Accession:O14521,Q53XW5, Q6IRW2, A6ND90, B3KQQ8, E9PIC0, E9PIG3 E9PQI9,
UniProt Related Accession:O14521
Molecular Weight:15,361 Da
NCBI Full Name:Succinate dehydrogenase
NCBI Synonym Full Names:succinate dehydrogenase complex subunit D
NCBI Official Symbol:SDHD  
NCBI Official Synonym Symbols:PGL; CBT1; CWS3; PGL1; QPs3; SDH4; cybS; CII-4  
NCBI Protein Information:succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
UniProt Protein Name:Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
UniProt Synonym Protein Names:CII-4; QPs3; Succinate dehydrogenase complex subunit D; Succinate-ubiquinone oxidoreductase cytochrome b small subunit; Succinate-ubiquinone reductase membrane anchor subunit
Protein Family:Succinate dehydrogenase
UniProt Gene Name:SDHD  
UniProt Entry Name:DHSD_HUMAN
Anti-SDHD Antibody (CAB16240)Western blot analysis of extracts of various cell lines, using SDHD antibody (CAB16240) at 1:3000 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: 10s.
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