Antibody Name:	Anti-HADH Antibody
Antibody SKU:	CAB1076
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-314 of human HADH (NP_005318.3).

Application:	WB IHC
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	HepG2, Mouse kidney, Mouse pancreas, Rat kidney, Rat liver

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-314 of human HADH (NP_005318.3).
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:	MAFV TRQF MRSV SSSS TASA SAKK IIVK HVTV IGGG LMGA GIAQ VAAA TGHT VVLV DQTE DILA KSKK GIEE SLRK VAKK KFAE NPKA GDEF VEKT LSTI ATST DAAS VVHS TDLV VEAI VENL KVKN ELFK RLDK FAAE HTIF ASNT SSLQ ITSI ANAT TRQD RFAG LHFF NPVP VMKL VEVI KTPM TSQK TFES LVDF SKAL GKHP VSCK DTPG FIVN RLLV PYLM EAIR LYER GDAS KEDI DTAM KLGA GYPM GPFE LLDY VGLD TTKF IVDG WHEM DAEN PLHQ PSPS LNKL VAEN KFGK KTGE GFYK YK
Gene ID:	3033
Uniprot:	Q16836
Cellular Location:	Mitochondrion matrix
Calculated MW:	34kDa/36kDa/42kDa
Observed MW:	34kDa

Synonyms:

HADH, HAD, HADH1, HADHSC, HCDH, HHF4, MSCHAD, SCHAD

Background:

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.

UniProt Protein Function:	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.
UniProt Protein Details:	Protein type:Lipid Metabolism - fatty acid elongation in mitochondria; Oxidoreductase; Amino Acid Metabolism - tryptophan; Amino Acid Metabolism - lysine degradation; Carbohydrate Metabolism - butanoate; EC 1.1.1.35; Lipid Metabolism - fatty acid; Amino Acid Metabolism - valine, leucine and isoleucine degradation Chromosomal Location of Human Ortholog: 4q22-q26 Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; cytoplasm; mitochondrial inner membrane Molecular Function:3-hydroxyacyl-CoA dehydrogenase activity Biological Process: response to drug; fatty acid beta-oxidation; cellular lipid metabolic process; response to activity; response to insulin stimulus; negative regulation of insulin secretion Disease: 3-hydroxyacyl-coa Dehydrogenase Deficiency; Hyperinsulinemic Hypoglycemia, Familial, 4
NCBI Summary:	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 Code:	Q16836
NCBI GenInfo Identifier:	311033442
NCBI Gene ID:	3033
NCBI Accession:	Q16836.3
UniProt Secondary Accession:	Q16836,O00324, O00397, O00753, Q4W5B4, J3KQ17,
UniProt Related Accession:	Q16836
Molecular Weight:	314
NCBI Full Name:	Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
NCBI 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; short-chain 3-hydroxyacyl-CoA dehydrogenase; L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain; medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase
UniProt Protein Name:	Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
UniProt Synonym Protein Names:	Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase
Protein Family:	Hydroxyacyl-coenzyme A dehydrogenase
UniProt Gene Name:	HADH
UniProt Entry Name:	HCDH_HUMAN

	Western blot analysis of extracts of various cell lines, using HADH antibody (CAB1076) 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 tonsil using HADH antibody (CAB1076) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded mouse brain using HADH antibody (CAB1076) at dilution of 1:100 (40x lens).

Antibodies	ELISA Kits
HADH Antibody (PACO09667)	Human HADH / Hydroxyacyl-coenzyme A dehydrogenase ELISA Kit
	Human Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial (HADH) ELISA Kit

Secondary Antibody

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