Product Name	CPT2
Chinese Name	肉毒碱棕榈酰基转移酶2Recombinant rabbit monoclonal anti
Alias	CPT2/CPT1; Carnitine O palmitoyltransferase 2; Carnitine O palmitoyltransferase 2 mitochondrial; Carnitine O-palmitoyltransferase 2; Carnitine palmitoyltransferase II; CPT 1; CPT 2; CPT II; CPT1; CPT2_HUMAN; CPTASE; CPTII; mitochondrial.
Research Area	Cardiovascular  Cell biology  immunology  Signal transduction  Lipoprotein  The new supersedes the old  Mitochondrion
Immunogen Species	Rabbit
Clonality	Monoclonal
Clone NO.	3B10
React Species	Human, Mouse,  (predicted: Rat, )
Applications	WB=1:500-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50 IF=1:50-200 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	71kDa
Cellular localization	cytoplasmic Mitochondrion
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human CPT2
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	The protein encoded by this gene is a nuclear protein which is transported to the mitochondrial inner membrane. Together with carnitine palmitoyltransferase I, the encoded protein oxidizes long-chain fatty acids in the mitochondria. Defects in this gene are associated with mitochondrial long-chain fatty-acid (LCFA) oxidation disorders. [provided by RefSeq, Jul 2008]. Subcellular Location: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side. DISEASE: Carnitine palmitoyltransferase 2 deficiency late-onset (CPT2D) [MIM:255110]: Autosomal recessive disorder characterized by recurrent myoglobinuria, episodes of muscle pain, stiffness, and rhabdomyolysis. These symptoms are triggered by prolonged exercise, fasting or viral infection and patients are usually young adults. In addition to this classical, late-onset, muscular type, a hepatic or hepatocardiomuscular form has been reported in infants. Clinical pictures in these children or neonates include hypoketotic hypoglycemia, liver dysfunction, cardiomyopathy and sudden death. Note=The disease is caused by mutations affecting the gene represented in this entry. Carnitine palmitoyltransferase 2 deficiency infantile (CPT2DI) [MIM:600649]: A disorder of mitochondrial long-chain fatty acid oxidation characterized by hepatic or hepato-cardio-muscular manifestations with onset in infancy. Clinical features include hypoketotic hypoglycemia, lethargy, seizures, hepatomegaly, liver dysfunction, cardiomegaly and dilated cardiomyopathy. Note=The disease is caused by mutations affecting the gene represented in this entry. Carnitine palmitoyltransferase 2 deficiency lethal neonatal (CPT2D-LN) [MIM:608836]: Lethal neonatal form of CPT2D. This rarely presentation is antenatal with cerebral periventricular cysts and cystic dysplastic kidneys. The clinical variability of the disease is likely attributed to the variable residual enzymatic activity. Note=The disease is caused by mutations affecting the gene represented in this entry. Encephalopathy, acute, infection-induced, 4 (IIAE4) [MIM:614212]: A severe neurologic complication of an infection. It manifests within days in otherwise healthy children after common viral infections, without evidence of viral infection of the brain or inflammatory cell infiltration. In affected children, high-grade fever is accompanied within 12 to 48 hours by febrile convulsions, often leading to coma, multiple-organ failure, brain edema, and high morbidity and mortality. The infections are usually viral, particularly influenza, although other viruses and even mycoplasma have been found to cause the disorder. Note=Disease susceptibility is associated with variations affecting the gene represented in this entry. CPT2 polymorphic variants do not cause classical carnitine palmitoyltransferase 2 deficiency, and patients harboring any of them are asymptomatic most of the time. However, they are prone to viral infection (high fever)-related encephalopathy (PubMed:21697855). Similarity: Belongs to the carnitine/choline acetyltransferase family. SWISS: P23786 Gene ID: 1376 Database links: Entrez Gene: 1376 Human Entrez Gene: 12896 Mouse Entrez Gene: 25413 Rat Omim: 600650 Human SwissProt: P23786 Human SwissProt: P52825 Mouse SwissProt: P18886 Rat Unigene: 713535 Human Unigene: 307620 Mouse Unigene: 11389 Rat
Product Picture	Sample: Lane 1: Hela cell lysate Lane 2: 293 cell lysate Lane 3: HepG2 cell lysate Lane 4: NIH/3T3 cell lysate Primary: Anti-CPT2 (SLM-52621R) at 1:500 dilution Secondary: Goat Anti-Rabbit IgG - HRP at 1:5000 dilution Predicted band size: 71 kD Observed band size: 71 kD Paraformaldehyde-fixed, paraffin embedded (mouse kidney); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (CPT2) Monoclonal Antibody, Unconjugated (SLM-52621R) at 1:50 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (human liver carcinoma); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (CPT2) Monoclonal Antibody, Unconjugated (SLM-52621R) at 1:50 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining. Paraformaldehyde-fixed, paraffin embedded (human kidney tissue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (CPT2) Monoclonal Antibody, Unconjugated (SLM-52621R) at 1:50 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining. HepG2 cell; 4% Paraformaldehyde-fixed; Triton X-100 at room temperature for 20 min; Blocking buffer (normal goat serum,C-0005) at 37°C for 20 min; Antibody incubation with (CPT2) monoclonal Antibody, Unconjugated (SLM-52621R) 1:50, 90 minutes at 37°C; followed by a conjugated Goat Anti-Rabbit IgG antibody at 37°C for 90 minutes, DAPI (blue, C02-04002) was used to stain the cell nuclei. HepG2 cell; 4% Paraformaldehyde-fixed; Triton X-100 at room temperature for 20 min; Blocking buffer (normal goat serum,C-0005) at 37°C for 20 min; Antibody incubation with (CPT2) monoclonal Antibody, Unconjugated (SLM-52621R) 1:50, 90 minutes at 37°C; followed by a conjugated Goat Anti-Rabbit IgG antibody at 37°C for 90 minutes, DAPI (blue, C02-04002) was used to stain the cell nuclei.

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