Antibody Protein ELISA Kit from MyBioSource header Telephone 1.858.633.0165          
1.855.MyBioSource
Fax 1.858.633.0166    Email sales@mybiosource.com 
tel/fax
menu1 Home Products Ordering Company Help menu7
bottom
   MyBioSource rightarrow Recombinant Protein rightarrow ACADVL  rightarrow LOG IN  rightarrow MY ACCOUNT  rightarrow CART CONTENTS  rightarrow CHECKOUT 
horiz bar
MyBioSource Menu separator
separator
ELISA Kit ELISA Kit
CLIA Kit CLIA Kit
PCR Kit PCR Kit
Monoclonal Antibody Monoclonal Antibody
Polyclonal Antibody Polyclonal Antibody
Secondary Antibody Secondary Antibody
Antigen Antigen
Biochemical Biochemical
cDNA Clone cDNA Clone
siRNA siRNA
Peptide Peptide
Recombinant/Purified Protein Rec./Purified Protein

Custom ELISA Kit Custom ELISA Kit
Custom Protein Custom Protein
Custom Antibody Custom Antibody
Antibody Matched Pairs Antibody Matched Pairs
Antibody & Corresponding Blocking Peptide Pairs Antibody Peptide Pairs
Phospho-Specific Antibodies Phospho Antibodies
Products by Disease Products by Disease
Products by Pathway Products by Pathway
Products by Tissue Products by Tissue

arrow Advanced Search
arrow Submit Technical Q&A
arrow International Distributors
arrow Contact Us
separator
Our Best Sellers moreseparator
separator
 • Vitamin K-Dependent Gamma-Carboxylase (GGCX) ELISA Kit
 • Vascular Endothelial Growth Factor 120AA (VEGF) Recombinant Protein
 • SSTR1 Antibody
 • HinfI Restriction Enzyme
 • SSTR2 Antibody
 • Gastrokine 2 (GKN2) ELISA Kit
 • Glucose 6 Phosphate (G6P) ELISA Kit
 • blca-4 ELISA Kit
 • AGTR1 cDNA Clone
 • Tail virion protein G7P (VII) Recombinant Protein
 • N-acyl homoserine lactonase AiiA (aiiA) Recombinant Protein
 • Cytokinin MB (CKMB) ELISA Kit
 • Lysozyme (E) Recombinant Protein
 • Histone H2A (H2AFZ) Antibody
 downarrow more ...
separator
separator
DatasheetFull DatasheetPrinter Friendly DatasheetPrint This DatasheetAdd to Compare ListHave Questions? Ask UsRequest for a Quotation today

ACADVL recombinant protein :: Acyl-CoA Dehydrogenase Recombinant Protein

Scan QR to view Datasheet Catalog #    MBS144138 ACADVL recombinant protein
Unit / Price
0.002 mg  /  $140 +1 FREE 8GB USB
0.01 mg  /  $205 +1 FREE 8GB USB
1 mg  /  $5,015 +6 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   

Acyl-CoA Dehydrogenase (ACADVL), Recombinant Protein

 Also Known As   

Recombinant Human Acyl-CoA Dehydrogenase, Very Long Chain

 Product Synonym Names    ACADVL Human; Acyl-CoA Dehydrogenase, Very Long Chain Human Recombinant; ACAD6; LCACD; VLCAD
 Product Gene Name   

ACADVL recombinant protein

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
Table BarTOPTable Bar
 Sequence    MGSSHHH HHH SSGLVPRGSH MAGGAA QLAL DKSDSHPSDA LTRKKPAKAE SKSFAVGMFK GQLTTDQVFP YPSVLNEEQT QFLKELVEPV SRFFEEVNDP AKNDALEMVE ETTWQGLKEL GAFGLQVPSE LGGVGLCNTQ YARLVEIVGM HDLGVGITLG AHQSIGFKGI LLFGTKAQKE KYLPKLASGE TVAAFCLTEP SSGSDAASIR TSAVPSPCGK YYTLNGSKLW ISNGGLADIF TVFAKTPVTD PATGAVKEKI TAFVVERGFG GITHGPPEKK MGIKASNTAE VFFDGVRVPS ENVLGEVGSG FKVAMHILNN GRFGMAAALA GTMRGIIAKA VDHATNRTQF GEKIHNFGLI QEKLARMVML QYVTESMAYM VSANMDQGAT DFQIEAAISK IFGSEAAWKV TDECIQIMGG MGFMKEPGVE RVLRDLRIFR IFEGTNDILR LFVALQGCMD KGKELSGLGS ALKNPFGNAG LLLGEAGKQL RRRAGLGSGL SLSGLVHPEL SRSGELAVRA LEQFATVVEA KLIKHKKGIV NEQFLLQRLA DGAIDLYAMV VVLSRASRSL SEGHPTAQHE KMLCDTWCIE AAARIREGMA ALQSDPWQQE LYRNFKSISK ALVERGGVVT SNPLGF
 OMIM    201475
 3D Structure    ModBase 3D Structure for P49748
Section Bar
 Host    E Coli
Section Bar
 Purity/Purification    Greater than 90.0% as determined by SDS-PAGE.
 Form/Format    The 0.5mg/ml protein solution contains 20mM Tris-HCl buffer pH-8, 1mM DTT, 1mM EDTA, 10% glycerol and 100mM NaCl.
Sterile Filtered clear solution.
Section Bar
Section Bar
 Preparation and Storage    Store ACADVL at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.
 Other Notes    Small volumes of ACADVL recombinant protein 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.
Table BarTOPTable Bar
 

Related Product Information for ACADVL recombinant protein

   Description: ACADVL Human Recombinant produced in E Coli is a non-glycosylated, polypeptide chain containing 636 amino acids (41-655 a.a.) and having a molecular mass of 68.5 kda. ACADVL contains 21 amino acid His-Tag at the N-terminus and is purified by proprietary chromatographic techniques.

Introduction: ACADVL is an inner mitochondrial membrane enzyme that is part of the family of acyl-CoA dehydrogenases. ACADVL protein participates in lipid metabolism and has catalytic activity toward esters of long chain and very long chain fatty acids such as palmitoyl-CoA and stearoyl-CoA, and is involved in the first step of the fatty acid beta-oxidation pathway. ACADVL deficiency in reduces myocardial fatty acid beta-oxidation and is related with cardiomyopathy.
 Product Categories/Family for ACADVL recombinant protein    ENZYMES; Enzymes; Dehydrogenase
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 GenBank Nucleotide #    NM_000018.3 [Other Products]
Section Bar
 UniProt Primary Accession #    P49748 [Other Products]
 UniProt Secondary Accession #    O76056; Q8WUL0; B4DEB6; F5H2A9 [Other Products]
 UniProt Related Accession #    P49748 [Other Products]
 Molecular Weight    72,927 Da
Table BarTOPTable Bar
 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; acyl-Coenzyme A dehydrogenase, very long chain
 UniProt Protein Name    Very long-chain specific acyl-CoA dehydrogenase, mitochondrial
 Protein Family    Acyl-CoA dehydrogenase
 UniProt Gene Name    ACADVL [Similar Products]
 UniProt Synonym Gene Names    VLCAD; VLCAD [Similar Products]
 UniProt Entry Name    ACADV_HUMAN
Section Bar
 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]
Section Bar
 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: Oxidoreductase; EC 1.3.8.9; Lipid Metabolism - fatty acid; Mitochondrial

Chromosomal Location of Human Ortholog: 17p13.1

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

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

Biological Process: unfolded protein response, activation of signaling protein activity; cellular protein metabolic process; fatty acid beta-oxidation; epithelial cell differentiation; unfolded protein response; cellular lipid metabolic process; thermoregulation; fatty acid beta-oxidation using acyl-CoA dehydrogenase; negative regulation of fatty acid biosynthetic process; negative regulation of fatty acid oxidation; energy derivation by oxidation of organic compounds

Disease: Acyl-coa Dehydrogenase, Very Long-chain, Deficiency Of
Table BarTOPTable Bar
 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.
Table BarTOPTable Bar
 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.
Table BarTOPTable Bar
Products associated with ACADVL recombinant proteinPathways associated with ACADVL recombinant protein
 Reference Product  PubMed Publications
 CPT1A recombinant protein  >8 publications with ACADVL and CPT1A
 CPT2 recombinant protein  >8 publications with ACADVL and CPT2
 CPT1B recombinant protein  >4 publications with ACADVL and CPT1B
 ACSL1 recombinant protein  >2 publications with ACADVL and ACSL1
 HADHA recombinant protein  >2 publications with ACADVL and HADHA
 HADHB recombinant protein  >1 publications with ACADVL and HADHB
 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
Diseases associated with ACADVL recombinant proteinOrgans/Tissues associated with ACADVL recombinant protein
 Disease Name  Pubmed Publications
 VLCAD deficiency Antibodies  >140 publications with ACADVL and VLCAD deficiency
 Mitochondrial Diseases Antibodies  >58 publications with ACADVL and Mitochondrial Diseases
 Fatty Liver Antibodies  >44 publications with ACADVL and Fatty Liver
 Cardiovascular Diseases Antibodies  >27 publications with ACADVL and Cardiovascular Diseases
 Liver Diseases Antibodies  >21 publications with ACADVL and Liver Diseases
 Hepatomegaly Antibodies  >12 publications with ACADVL and Hepatomegaly
 Disease Models, Animal Antibodies  >9 publications with ACADVL and Disease Models, Animal
 Hypertrophy Antibodies  >8 publications with ACADVL and Hypertrophy
 Obesity Antibodies  >6 publications with ACADVL and Obesity
 Inflammation Antibodies  >3 publications with ACADVL and Inflammation
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >69 publications with ACADVL and Blood
 Muscle Antibodies  >62 publications with ACADVL and Muscle
 Liver Antibodies  >50 publications with ACADVL and Liver
 Heart Antibodies  >30 publications with ACADVL and Heart
 Skin Antibodies  >14 publications with ACADVL and Skin
 Adipose Tissue Antibodies  >9 publications with ACADVL and Adipose Tissue
 Brain Antibodies  >7 publications with ACADVL and Brain
 Pancreas Antibodies  >5 publications with ACADVL and Pancreas
 Nerve Antibodies  >4 publications with ACADVL and Nerve
 Connective Tissue Antibodies  >4 publications with ACADVL and Connective Tissue
Table BarTOPTable Bar
horiz bar
 SSL   Follow us on Facebook Follow us onTwitter Follow us on Google Plus Connect us on LinkedIn Subscribe to our RSS Feed for latest products and special promotions