Anti-HMGCS2 항체 (CAB14244)

Antibody Name:	Anti-HMGCS2 Antibody
Antibody SKU:	CAB14244
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 269-508 of human HMGCS2 (NP_005509.1).

Application:	WB IHC IF
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:100 IF 1:50 - 1:100
Reactivity:	Human, Mouse, Rat
Positive Samples:	Mouse heart, Mouse liver, Rat kidney

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 269-508 of human HMGCS2 (NP_005509.1).
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:	CYTS YRKK IQNQ WKQA GSDR PFTL DDLQ YMIF HTPF CKMV QKSL ARLM FNDF LSAS SDTQ TSLY KGLE AFGG LKLE DTYT NKDL DKAL LKAS QDMF DKKT KASL YLST HNGN MYTS SLYG CLAS LLSH HSAQ ELAG SRIG AFSY GSGL AASF FSFR VSQD AAPG SPLD KLVS STSD LPKR LASR KCVS PEEF TEIM NQRE QFYH KVNF SPPG DTNS LFPG TWYL ERVD EQHR RKYA RRPV
Gene ID:	3158
Uniprot:	P54868
Cellular Location:	Mitochondrion
Calculated MW:	50kDa/52kDa/56kDa
Observed MW:	50kDa

Synonyms:

HMGCS2

Background:

The protein encoded by this gene belongs to the HMG-CoA synthase family. It is a mitochondrial enzyme that catalyzes the first reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting. Mutations in this gene are associated with HMG-CoA synthase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

UniProt Protein Function:	HMGCS2: This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase. Defects in HMGCS2 are the cause of HMG-CoA synthase deficiency (HMGCS deficiency); also known as deficiency of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase 2. Affected individuals present with severe hypoketotic hypoglycemia, mild hepatomegaly, or fatty liver, and a nondiagnostic pattern of urinary organic acids with increase of medium and short chain dicarboxylic acids. Belongs to the HMG-CoA synthase family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:Transferase; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 2.3.3.10; Carbohydrate Metabolism - butanoate; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Lipid Metabolism - synthesis and degradation of ketone bodies Chromosomal Location of Human Ortholog: 1p13-p12 Cellular Component: mitochondrial matrix; mitochondrion Molecular Function:hydroxymethylglutaryl-CoA synthase activity Biological Process: ketone body biosynthetic process Disease: 3-hydroxy-3-methylglutaryl-coa Synthase 2 Deficiency
NCBI Summary:	The protein encoded by this gene belongs to the HMG-CoA synthase family. It is a mitochondrial enzyme that catalyzes the first reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting. Mutations in this gene are associated with HMG-CoA synthase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2009]
UniProt Code:	P54868
NCBI GenInfo Identifier:	1708234
NCBI Gene ID:	3158
NCBI Accession:	P54868.1
UniProt Secondary Accession:	P54868,Q5SZU2, Q6IBF4, B7Z8R3, D3Y5K6,
UniProt Related Accession:	P54868
Molecular Weight:	50,050 Da
NCBI Full Name:	Hydroxymethylglutaryl-CoA synthase, mitochondrial
NCBI Synonym Full Names:	3-hydroxy-3-methylglutaryl-CoA synthase 2
NCBI Official Symbol:	HMGCS2
NCBI Protein Information:	hydroxymethylglutaryl-CoA synthase, mitochondrial
UniProt Protein Name:	Hydroxymethylglutaryl-CoA synthase, mitochondrial
UniProt Synonym Protein Names:	3-hydroxy-3-methylglutaryl coenzyme A synthase
UniProt Gene Name:	HMGCS2
UniProt Entry Name:	HMCS2_HUMAN

	Western blot analysis of extracts of various cell lines, using HMGCS2 antibody (CAB14244) 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: 30s.
	Immunohistochemistry of paraffin-embedded human thyroid cancer using HMGCS2 antibody (CAB14244) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded human colon carcinoma using HMGCS2 antibody (CAB14244) at dilution of 1:100 (40x lens).
	Immunofluorescence analysis of rat liver using HMGCS2 Polyclonal Antibody (CAB14244) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
	Immunofluorescence analysis of human liver using HMGCS2 Polyclonal Antibody (CAB14244) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.

Secondary Antibody

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