Mouse anti-Human HADHSC Monoclonal Antibody | anti-HADHSC antibody

HADHSC (L-3-Hydroxyacyl Coenzyme A Dehydrogenase Short Chain, Hydroxyacyl-coenzyme A Dehydrogenase Mitochondrial, HAD, HADH, HADH1, HCDH, HHF4, Medium and Short Chain L-3-Hydroxyacyl-coenzyme A Dehydrogenase, M/SCHAD, MGC8392, Short Chain 3-Hydroxyacyl-Co

Cat. Num. AAA6147523

• Gene Names: HADH; HAD; HCDH; HHF4; HADH1; SCHAD; HADHSC; MSCHAD
• Reactivity: Human
• Applications: ELISA, Immunofluorescence, Immunohistochemistry, Immunoprecipitation, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: HADHSC; Monoclonal Antibody; HADHSC (L-3-Hydroxyacyl Coenzyme A Dehydrogenase Short Chain; Hydroxyacyl-coenzyme A Dehydrogenase Mitochondrial; HAD; HADH; HADH1; HCDH; HHF4; Medium and Short Chain L-3-Hydroxyacyl-coenzyme A Dehydrogenase; M/SCHAD; MGC8392; Short Chain 3-Hydroxyacyl-Co; anti-HADHSC antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2b,k
• Clone Number: 4B5
• Specificity: Recognizes human HADHSC.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein isothiocyanate (FITC).

• Applicable Applications for anti-HADHSC antibody: ELISA (EIA), Immunofluorescence (IF), Immunohistochemistry (IHC), Immunoprecipitation (IP), Western Blot (WB)
• Application Notes: IF: 10ug/ml; Applications are based on unconjugated antibody.
• Immunogen: Partial recombinant corresponding to aa205-314 from human HADHSC (NP_005318) with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: GKHPVSCKDTPGFIVNRLLVPYLMEAIRLYERGDASKEDIDTAMKLGAGYPMGPFELLDYVGLDTTKFIVDGWHEMDAENPLHQPSPSLNKLVAENKFGKKTGEGFYKYK
• Conjugate: FITC
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (37.84kD).)

Western Blot (WB)

(HADHSC monoclonal antibody Western Blot analysis of HADHSC expression in HepG2.)

Western Blot (WB)

(Western Blot analysis of HADH expression in transfected 293T cell line by HADHSC monoclonal antibody. Lane 1: HADH transfected lysate (34.3kD). Lane 2: Non-transfected lysate.)

Immunohistochemistry (IHC)

(Immunoperoxidase of monoclonal antibody to HADHSC on formalin-fixed paraffin-embedded human colon. [antibody concentration 3ug/ml])

Immunofluorescence (IF)

(Immunofluorescence of monoclonal antibody to HADHSC on HeLa cell. [antibody concentration 10ug/ml])

Immunoprecipitation (IP)

(Immunoprecipitation of HADH transfected lysate using anti-HADH monoclonal antibody and Protein A Magnetic Bead, and immunoblotted with HADH rabbit polyclonal antibody.)

Testing Data

(Detection limit for recombinant GST tagged HADHSC is ~0.03ng/ml as a capture antibody.)

Product Categories/Family for anti-HADHSC antibody

Antibodies; Enzymes; Dehydrogenase

NCBI and Uniprot Product Information

• NCBI GI #: 296179429
• NCBI GeneID: 3033
• NCBI Accession #: NP_005318
• NCBI GenBank Nucleotide #: NM_005327
• UniProt Accession #: Q16836; O00324; O00397; O00753; Q4W5B4; J3KQ17
• Molecular Weight: 36,051 Da
• NCBI Official Full Name: hydroxyacyl-coenzyme A dehydrogenase, mitochondrial isoform 2
• NCBI Official Synonym Full Names: hydroxyacyl-CoA dehydrogenase
• NCBI Official Symbol: HADH
• NCBI Official Synonym Symbols: HAD; HCDH; HHF4; HADH1; SCHAD; HADHSC; MSCHAD
• NCBI Protein Information: hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain; medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; short-chain 3-hydroxyacyl-CoA dehydrogenase
• UniProt Protein Name: Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
• UniProt Gene Name: HADH
• UniProt Synonym Gene Names: HAD; HADHSC; SCHAD; HCDH
• UniProt Entry Name: HCDH_HUMAN

Uniprot Description

HADHSC: Plays an essential role in the mitochondrial beta- oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA. Defects in HADH are the cause of 3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency). HADH deficiency is a metabolic disorder with various clinical presentations including hypoglycemia, hepatoencephalopathy, myopathy or cardiomyopathy, and in some cases sudden death. Defects in HADH are the cause of familial hyperinsulinemic hypoglycemia type 4 (HHF4); also known as persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or congenital hyperinsulinism. HHF is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. It causes nesidioblastosis, a diffuse abnormality of the pancreas in which there is extensive, often disorganized formation of new islets. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur. HHF4 should be easily recognizable by analysis of acylcarnitine species and that this disorder responds well to treatment with diazoxide. It provides the first 'experiment of nature' that links impaired fatty acid oxidation to hyperinsulinism and that provides support for the concept that a lipid signaling pathway is implicated in the control of insulin secretion. Belongs to the 3-hydroxyacyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Amino Acid Metabolism - lysine degradation; Lipid Metabolism - fatty acid elongation in mitochondria; Carbohydrate Metabolism - butanoate; Oxidoreductase; Lipid Metabolism - fatty acid; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Amino Acid Metabolism - tryptophan; EC 1.1.1.35

Chromosomal Location of Human Ortholog: 4q22-q26

Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; mitochondrial inner membrane; cytoplasm

Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity

Biological Process: response to drug; fatty acid beta-oxidation; response to activity; cellular lipid metabolic process; negative regulation of insulin secretion; response to insulin stimulus

Disease: 3-hydroxyacyl-coa Dehydrogenase Deficiency; Hyperinsulinemic Hypoglycemia, Familial, 4