Human Hydroxyacyl Coenzyme A Dehydrogenase (HADH) ELISA Kit | HADH elisa kit

Human Hydroxyacyl Coenzyme A Dehydrogenase (HADH) ELISA Kit

Cat. Num. AAA2122030

• Gene Names: HADH; HAD; HCDH; HHF4; HADH1; SCHAD; HADHSC; MSCHAD
• Reactivity: Human
• Synonyms: Hydroxyacyl Coenzyme A Dehydrogenase (HADH); Human Hydroxyacyl Coenzyme A Dehydrogenase (HADH) ELISA Kit; HADHSC; HADH1; SCHAD; L-3-Hydroxyacyl-Coenzyme A Dehydrogenase; Short Chain; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; HADH elisa kit

• Reactivity: Human
• Specificity: This assay has high sensitivity and excellent specificity for detection of Hydroxyacyl Coenzyme A Dehydrogenase (HADH).; No significant cross-reactivity or interference between Hydroxyacyl Coenzyme A Dehydrogenase (HADH) and analogues was observed.
• Sequence Length: 314

• Assay Type: Double-antibody Sandwich
• Sample: Tissue Homogenates, Cell Lysates and Other Biological Fluids
• Dectection Range: 0.312-20ng/mL
• Sensitivity: The minimum detectable dose of this kit is typically less than 0.119ng/mL
• Precision: Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level Hydroxyacyl Coenzyme A Dehydrogenase (HADH) were tested 20 times on one plate, respectively.; Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level Hydroxyacyl Coenzyme A Dehydrogenase (HADH) were tested on 3 different plates, 8 replicates in each plate.; CV(%) = SD/meanX100; Intra-Assay: CV<10%; Inter-Assay: CV<12%
• Preparation and Storage: The stability of kit is determined by the loss rate of activity. The loss rate of this kit is less than 5% within the expiration date under appropriate storage condition.; To minimize extra influence on the performance, operation procedures and lab conditions, especially room temperature, air humidity, incubator temperature should be strictly controlled. It is also strongly suggested that the whole assay is performed by the same operator from the beginning to the end.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for HADH elisa kit

The test principle applied in this kit is Sandwich enzyme immunoassay. The microtiter plate provided in this kit has been pre-coated with an antibody specific to Hydroxyacyl Coenzyme A Dehydrogenase (HADH). Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody specific to Hydroxyacyl Coenzyme A Dehydrogenase (HADH). Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. After TMB substrate solution is added, only those wells that contain Hydroxyacyl Coenzyme A Dehydrogenase (HADH), biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450nm +/- 10nm. The concentration of Hydroxyacyl Coenzyme A Dehydrogenase (HADH) in the samples is then determined by comparing the O.D. of the samples to the standard curve.

Product Categories/Family for HADH elisa kit

Enzyme & Kinase

NCBI and Uniprot Product Information

• NCBI GI #: 296179429
• NCBI GeneID: 3033
• NCBI Accession #: NP_005318.3
• NCBI GenBank Nucleotide #: NP_005318.3
• UniProt Accession #: Q16836
• 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
• UniProt Protein Name: Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
• Protein Family: Hydroxyacyl-coenzyme A dehydrogenase
• UniProt Gene Name: HADH
• UniProt Synonym Gene Names: HAD; HADHSC; SCHAD; HCDH
• UniProt Entry Name: HCDH_HUMAN

NCBI Description

This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. The encoded protein functions in the mitochondrial matrix to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids. Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemia. The human genome contains a related pseudogene of this gene on chromosome 15. [provided by RefSeq, May 2010]

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