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ACADVL peptide :: VLCAD Peptide

Scan QR to view Datasheet Catalog #    MBS427218 ACADVL peptide
Unit / Price
0.1 mg  /  $190 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

VLCAD (ACADVL), Peptide

 Also Known As   

VLCAD Immunizing Peptide

 Product Synonym Names    ACADVL; acyl-Coenzyme A dehydrogenase, very long chain; ACAD6; LCACD; VLCAD
 Product Gene Name   

ACADVL peptide

[Similar Products]
 Product Synonym Gene Name    VLCAD [Similar Products]
 Antibody/Peptide Pairs    VLCAD peptide (MBS427218) is used for blocking the activity of VLCAD antibody (MBS422310)
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Sequence    DKSDSHPSDA LTRK-C
 OMIM    201475
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 Species Reactivity    Human
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 Form/Format    100ug of dried peptide
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 Preparation and Storage    Shipped at ambient temperature, store at -20 degree C
 ISO Certification    Manufactured in an ISO 9001:2015 Certified Laboratory.
 Other Notes    Small volumes of ACADVL peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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NCBI/Uniprot data below describe general gene information for ACADVL. It may not necessarily be applicable to this product.
 NCBI GI #    4557235
 NCBI GeneID    37
 NCBI Accession #    NP_000009.1 [Other Products]
 NCBI Related Accession #    NP_001029031.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_000018.3 [Other Products]
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 UniProt Secondary Accession #    O76056; Q8WUL0; B4DEB6; F5H2A9 [Other Products]
 UniProt Related Accession #    P49748 [Other Products]
 Molecular Weight    72,927 Da
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 NCBI Official Full Name    very long-chain specific acyl-CoA dehydrogenase, mitochondrial isoform 1
 NCBI Official Synonym Full Names    acyl-CoA dehydrogenase, very long chain
 NCBI Official Symbol    ACADVL [Similar Products]
 NCBI Official Synonym Symbols   
ACAD6; LCACD; VLCAD
[Similar Products]
 NCBI Protein Information    very long-chain specific acyl-CoA dehydrogenase, mitochondrial
 UniProt Protein Name    Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
 Protein Family    Very long-chain specific acyl-CoA dehydrogenase
 UniProt Gene Name    ACADVL [Similar Products]
 UniProt Synonym Gene Names    VLCAD; VLCAD [Similar Products]
 UniProt Entry Name    ACADV_HUMAN
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 NCBI Summary for ACADVL    The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]
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 UniProt Comments for ACADVL    ACADVL: Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons. Defects in ACADVL are the cause of acyl-CoA dehydrogenase very long chain deficiency (ACADVLD). ACADVLD is an autosomal recessive disease which leads to impaired long-chain fatty acid beta-oxidation. It is clinically heterogeneous, with three major phenotypes: a severe childhood form, with early onset, high mortality, and high incidence of cardiomyopathy; a milder childhood form, with later onset, usually with hypoketotic hypoglycemia as the main presenting feature, low mortality, and rare cardiomyopathy; and an adult form, with isolated skeletal muscle involvement, rhabdomyolysis, and myoglobinuria, usually triggered by exercise or fasting. Belongs to the acyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Mitochondrial; Lipid Metabolism - fatty acid; Oxidoreductase; EC 1.3.8.9

Chromosomal Location of Human Ortholog: 17p13.1

Cellular Component: cytoplasm; mitochondrial inner membrane; mitochondrial matrix; mitochondrion; nucleolus; nucleus

Molecular Function: acyl-CoA binding; acyl-CoA dehydrogenase activity; electron carrier activity; FAD binding; long-chain-acyl-CoA dehydrogenase activity; very-long-chain-acyl-CoA dehydrogenase activity

Biological Process: energy derivation by oxidation of organic compounds; epithelial cell differentiation; fatty acid beta-oxidation; fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis; negative regulation of fatty acid biosynthetic process; negative regulation of fatty acid oxidation; thermoregulation; very-long-chain fatty acid catabolic process

Disease: Acyl-coa Dehydrogenase, Very Long-chain, Deficiency Of
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 Research Articles on ACADVL    1. 11 mutations in ACADVL gene in 7 patients, 7 reported (p.S22X, p.W427X, p.A213T, p.G222R, p.R450H, c.296-297delCA, c.1605+1G>T), 4 novel (p.S72F, p.Q100X, p.M437T, p.D466Y). p.R450H and p.D466Y (14.28%, 2/14 alleles) mutations identified in 2 alleles.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Pathways associated with ACADVL peptideDiseases associated with ACADVL peptide
 Products by Pathway  Pathway Diagram
 Beta Oxidation Of Palmitoyl-CoA To Myristoyl-CoA Pathway antibodies  Beta Oxidation Of Palmitoyl-CoA To Myristoyl-CoA Pathway Diagram
 FOXA2 And FOXA3 Transcription Factor Networks Pathway antibodies  FOXA2 And FOXA3 Transcription Factor Networks Pathway Diagram
 Fatty Acid Beta Oxidation Pathway antibodies  Fatty Acid Beta Oxidation Pathway Diagram
 Fatty Acid Degradation Pathway antibodies  Fatty Acid Degradation Pathway Diagram
 Fatty Acid Degradation Pathway antibodies  Fatty Acid Degradation Pathway Diagram
 Fatty Acid Metabolism Pathway antibodies  Fatty Acid Metabolism Pathway Diagram
 Fatty Acid Metabolism Pathway antibodies  Fatty Acid Metabolism Pathway Diagram
 Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway antibodies  Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway Diagram
 IRE1alpha Activates Chaperones Pathway antibodies  IRE1alpha Activates Chaperones Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Disease Name  Pubmed Publications
 VLCAD deficiency Antibodies  >163 publications with ACADVL and VLCAD deficiency
 Fatty Liver Antibodies  >50 publications with ACADVL and Fatty Liver
 Cardiovascular Diseases Antibodies  >32 publications with ACADVL and Cardiovascular Diseases
 Liver Diseases Antibodies  >27 publications with ACADVL and Liver Diseases
 Hepatomegaly Antibodies  >13 publications with ACADVL and Hepatomegaly
 Disease Models, Animal Antibodies  >10 publications with ACADVL and Disease Models, Animal
 Hypertrophy Antibodies  >9 publications with ACADVL and Hypertrophy
 Congenital Abnormalities Antibodies  >5 publications with ACADVL and Congenital Abnormalities
 Insulin Resistance Antibodies  >4 publications with ACADVL and Insulin Resistance
 Kidney Diseases Antibodies  >3 publications with ACADVL and Kidney Diseases
Organs/Tissues associated with ACADVL peptide
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >79 publications with ACADVL and Blood
 Muscle Antibodies  >67 publications with ACADVL and Muscle
 Liver Antibodies  >57 publications with ACADVL and Liver
 Heart Antibodies  >32 publications with ACADVL and Heart
 Skin Antibodies  >16 publications with ACADVL and Skin
 Adipose Tissue Antibodies  >9 publications with ACADVL and Adipose Tissue
 Brain Antibodies  >8 publications with ACADVL and Brain
 Pancreas Antibodies  >6 publications with ACADVL and Pancreas
 Kidney Antibodies  >5 publications with ACADVL and Kidney
 Bone Antibodies  >5 publications with ACADVL and Bone
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