Rabbit anti-Human CBT1 / SDHD Polyclonal Antibody | anti-CBT1 antibody

Rabbit Polyclonal to Human CBT1 / SDHD

Cat. Num. AAA248258

• Gene Names: SDHD; PGL; CBT1; CWS3; PGL1; QPs3; SDH4; cybS; CII-4
• Reactivity: Human
• Applications: Immunohistochemistry, Western Blot
• Purity: Immunoaffinity Purified
• Synonyms: CBT1 / SDHD; Polyclonal Antibody; Rabbit Polyclonal to Human CBT1 / SDHD; Anti-CBT1 / SDHD Antibody (Internal) IHC-plus; SDHD; CBT1; CII-4; PGL; SDH4; QPs3; CybS; PGL1; Human CBT1; anti-CBT1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: Human SDHD. At least three isoforms of THBS are known to exist; this antibody will detect all three.
• Purity/Purification: Immunoaffinity Purified
• Form/Format: PBS, 0.02% sodium azide
• Concentration: 1 mg/ml (varies by lot)
• Sequence Length: 159

• Applicable Applications for anti-CBT1 antibody: Immunohistochemistry (IHC - Paraffin), Western Blot (WB)
• Application Notes: IHC-P (2.5 ug/ml), WB (1 - 2 ug/ml); Usage: SDHD antibody can be used for detection of SDHD by Western blot at 1-2 ug/ml.
• Target Species: Human
• Immunogen Description: A 15 amino acid peptide near the center of human SDHD.
• Immunogen Type: Synthetic peptide
• Immunogen: CBT1 / SDHD antibody was raised against a 15 amino acid peptide near the center of human SDHD.
• Antigen Modification: Internal
• Preparation and Storage: Store at -20 degree C. Aliquot to avoid freeze/thaw cycles.

Immunohistochemistry (IHC)

(Anti-CBT1 / SDHD antibody IHC staining of human kidney. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval.)

Immunofluorescence (IF)

(Immunofluorescence of SDHD in EL4 cells with SDHD antibody at 20 ug/ml.)

Immunocytochemistry (ICC)

(Immunocytochemistry of SDHD in EL4 cells with SDHD antibody at 2.5 ug/ml.)

Western Blot (WB)

(Western blot analysis of SDHD in EL4 cell lysate with SDHD antibody at (A) 1 and (B) 2 ug/ml.)

NCBI and Uniprot Product Information

• NCBI GI #: 4506865
• NCBI GeneID: 6392
• NCBI Accession #: NP_002993.1
• NCBI GenBank Nucleotide #: NM_003002.3
• UniProt Accession #: O14521; Q53XW5; Q6IRW2; A6ND90; B3KQQ8; E9PIC0; E9PIG3; E9PQI9
• Molecular Weight: 15,361 Da
• NCBI Official Full Name: succinate dehydrogenase
• NCBI Official Synonym Full Names: succinate dehydrogenase complex, subunit D, integral membrane protein
• 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; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial; succinate-ubiquinone reductase membrane anchor subunit; succinate-ubiquinone oxidoreductase cytochrome b small subunit
• UniProt Protein Name: Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
• Protein Family: Cytochrome b termination protein
• UniProt Gene Name: SDHD
• UniProt Synonym Gene Names: SDH4; CybS
• UniProt Entry Name: DHSD_HUMAN

NCBI Description

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 Description

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.

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

Chromosomal Location of Human Ortholog: 11q23

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

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

Biological Process: cellular metabolic process; tricarboxylic acid cycle

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