Anti-ACADSB 항체 (CAB15019)

Antibody Name:	Anti-ACADSB Antibody
Antibody SKU:	CAB15019
Antibody Size:	20uL, 50uL, 100uL
Application:	IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 133-432 of human ACADSB (NP_001600.1).

Application:	IHC IF
Recommended Dilution:	IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 133-432 of human ACADSB (NP_001600.1).
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:	ASVA VFCE IQNT LINT LIRK HGTE EQKA TYLP QLTT EKVG SFCL SEAG AGSD SFAL KTRA DKEG DYYV LNGS KMWI SSAE HAGL FLVM ANVD PTIG YKGI TSFL VDRD TPGL HIGK PENK LGLR ASST CPLT FENV KVPE ANIL GQIG HGYK YAIG SLNE GRIG IAAQ MLGL AQGC FDYT IPYI KERI QFGK RLFD FQGL QHQV AHVA TQLE AARL LTYN AARL LEAG KPFI KEAS MAKY YASE IAGQ TTSK CIEW MGGV GYTK DYPV EKYF RDAK IGTI YEGA SNIQ LNTI AKHI DAEY
Gene ID:	36
Uniprot:	P45954
Cellular Location:	Mitochondrion matrix
Calculated MW:	36kDa/47kDa
Observed MW:

Synonyms:

ACADSB, 2-MEBCAD, ACAD7, SBCAD

Background:

Short/branched chain acyl-CoA dehydrogenase(ACADSB) is a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. Substrate specificity is the primary characteristic used to define members of this gene family. The ACADSB gene product has the greatest activity towards the short branched chain acyl-CoA derivative, (S)-2-methylbutyryl-CoA, but also reacts significantly with other 2-methyl branched chain substrates and with short straight chain acyl-CoAs. The cDNA encodes for a mitochondrial precursor protein which is cleaved upon mitochondrial import and predicted to yield a mature peptide of approximately 43.7-KDa.

UniProt Protein Function:	ACADSB: Has greatest activity toward short branched chain acyl- CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl- CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent. Defects in ACADSB are the cause of short/branched-chain acyl-CoA dehydrogenase deficiency (SBCADD); also known as 2-methylbutyryl-CoA dehydrogenase deficiency or 2- methylbutyryl glycinuria. SBCADD is an autosomal recessive disorder and consists of a defect in catabolism of L-isoleucine which is characterized by an increase of 2-methylbutyrylglycine and 2-methylbutyrylcarnitine in blood and urine. Affected individuals have seizures and psychomotor delay as the main clinical features. Belongs to the acyl-CoA dehydrogenase family.
UniProt Protein Details:	Protein type:Oxidoreductase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 1.3.8.5; Lipid Metabolism - fatty acid; Mitochondrial Chromosomal Location of Human Ortholog: 10q26.13 Cellular Component: mitochondrion; mitochondrial matrix Molecular Function:acyl-CoA dehydrogenase activity; FAD binding Biological Process: branched chain family amino acid catabolic process; fatty acid metabolic process Disease: 2-methylbutyryl-coa Dehydrogenase Deficiency
NCBI Summary:	Short/branched chain acyl-CoA dehydrogenase(ACADSB) is a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. Substrate specificity is the primary characteristic used to define members of this gene family. The ACADSB gene product has the greatest activity towards the short branched chain acyl-CoA derivative, (S)-2-methylbutyryl-CoA, but also reacts significantly with other 2-methyl branched chain substrates and with short straight chain acyl-CoAs. The cDNA encodes for a mitochondrial precursor protein which is cleaved upon mitochondrial import and predicted to yield a mature peptide of approximately 43.7-KDa. [provided by RefSeq, Jul 2008]
UniProt Code:	P45954
NCBI GenInfo Identifier:	1168283
NCBI Gene ID:	36
NCBI Accession:	P45954.1
UniProt Related Accession:	P45954
Molecular Weight:
NCBI Full Name:	Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
NCBI Synonym Full Names:	acyl-CoA dehydrogenase short/branched chain
NCBI Official Symbol:	ACADSB
NCBI Official Synonym Symbols:	ACAD7; SBCAD; 2-MEBCAD
NCBI Protein Information:	short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
UniProt Protein Name:	Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
UniProt Synonym Protein Names:	2-methyl branched chain acyl-CoA dehydrogenase; 2-MEBCAD; 2-methylbutyryl-coenzyme A dehydrogenase; 2-methylbutyryl-CoA dehydrogenase
UniProt Gene Name:	ACADSB
UniProt Entry Name:	ACDSB_HUMAN

	Immunohistochemistry of paraffin-embedded rat brain using ACADSB antibody (CAB15019) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded human breast cancer using ACADSB antibody (CAB15019) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded mouse spinal cord using ACADSB antibody (CAB15019) at dilution of 1:100 (40x lens).
	Immunofluorescence analysis of HeLa cells using ACADSB antibody (CAB15019) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.

Antibodies

ACADSB Antibody (PACO07546)

Secondary Antibody

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