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Index > Protein center > ACADVL(Gene name) > Human
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  • ACADVL (Gene name),
  • Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (Protein name ),  ACADV_HUMAN from NCBI database.
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  • General Annotation
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  • Antigen Annotation
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  • 3D
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  • Predicted Eptitope
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  • Vaild Sequence
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  • Gene name:
    ACADVL(VLCAD);
    Protein name:
    Very long-chain specific acyl-CoA dehydrogenase, mitochondrial(VLCAD);
    Alternative:

    Organism:
    Human (Homo sapiens). 
    General Annotation
    Sub Unit:
    Homodimer.
    Function:
    Active toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accomodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.
    Subcellular Location:
    Mitochondrion inner membrane
    Protein Attributes:
    Sequence length:
    655
    Sequence:
    50:
    MQAARMAASL | GRQLLRLGGG | SSRLTALLGQ | PRPGPARRPY | AGGAAQLALD | 
    100:
    KSDSHPSDAL | TRKKPAKAES | KSFAVGMFKG | QLTTDQVFPY | PSVLNEEQTQ | 
    150:
    FLKELVEPVS | RFFEEVNDPA | KNDALEMVEE | TTWQGLKELG | AFGLQVPSEL | 
    200:
    GGVGLCNTQY | ARLVEIVGMH | DLGVGITLGA | HQSIGFKGIL | LFGTKAQKEK | 
    250:
    YLPKLASGET | VAAFCLTEPS | SGSDAASIRT | SAVPSPCGKY | YTLNGSKLWI | 
    300:
    SNGGLADIFT | VFAKTPVTDP | ATGAVKEKIT | AFVVERGFGG | ITHGPPEKKM | 
    350:
    GIKASNTAEV | FFDGVRVPSE | NVLGEVGSGF | KVAMHILNNG | RFGMAAALAG | 
    400:
    TMRGIIAKAV | DHATNRTQFG | EKIHNFGLIQ | EKLARMVMLQ | YVTESMAYMV | 
    450:
    SANMDQGATD | FQIEAAISKI | FGSEAAWKVT | DECIQIMGGM | GFMKEPGVER | 
    500:
    VLRDLRIFRI | FEGTNDILRL | FVALQGCMDK | GKELSGLGSA | LKNPFGNAGL | 
    550:
    LLGEAGKQLR | RRAGLGSGLS | LSGLVHPELS | RSGELAVRAL | EQFATVVEAK | 
    600:
    LIKHKKGIVN | EQFLLQRLAD | GAIDLYAMVV | VLSRASRSLS | EGHPTAQHEK | 
    650:
    MLCDTWCIEA | AARIREGMAA | LQSDPWQQEL | YRNFKSISKA | LVERGGVVTS | 
    655:
    NPLGF
    3D Structure:
    N/A
    Predicted Eptitope:
    Please Sign in.
    EIAab Sequence  Vaild Sequence:
    Please Sign in.
    Related Databases
    SMR:
    Pfam:
    KEGG:
    UniGene:
    String:
    MIM:
    Uniprot:
     
    FOR
    ELISA Kit for Human VLCAD
    ELISA Kit for Human VLCAD
    ELISA Kit for Human VLCAD
    ELISA Kit for Human VLCAD
    CLIA Kit for Human VLCAD
    CLIA Kit for Human VLCAD
    CLIA Kit for Human VLCAD
    CLIA Kit for Human VLCAD
    Polyclonal Antibody for Human VLCAD
    Polyclonal Antibody for Human VLCAD
    Polyclonal Antibody for Human VLCAD
    Polyclonal Antibody for Human VLCAD
    Monoclonal Antibody for Human VLCAD
    Monoclonal Antibody for Human VLCAD
    Monoclonal Antibody for Human VLCAD
    Monoclonal Antibody for Human VLCAD
    Protein for Human VLCAD
    Protein for Human VLCAD
    Protein for Human VLCAD
    Protein for Human VLCAD

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    Precision
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    Recovery
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    Linearity
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    References
    1. 1.
      "Cloning of human very-long-chain acyl-coenzyme A dehydrogenase and molecular characterization of its deficiency in two patients."
      Aoyama T. , Souri M. , Ueno I. , Kamijo T. , Yamaguchi S. , Rhead W.J. , Tanaka K. , Hashimoto T.
      Am. J. Hum. Genet.57:273-283(1995) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1)
    2. 2.
      "Cloning and characterization of human very-long-chain acyl-CoA dehydrogenase cDNA, chromosomal assignment of the gene and identification in four patients of nine different mutations within the VLCAD gene."
      Andresen B.S. , Bross P. , Vianey-Saban C. , Divry P. , Zabot M.-T. , Roe C.R. , Nada M.A. , Byskov A. , Kruse T.A. , Neve S. , Kristiansen K. , Knudsen I. , Corydon M.J. , Gregersen N.
      Hum. Mol. Genet.5:461-472(1996) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1);TISSUE SPECIFICITY;VARIANTS
      tissue: Placenta.
    3. 3.
      "Genomic DNA organization of human mitochondrial very-long-chain acyl-CoA dehydrogenase and mutation analysis."
      Orii K.O. , Aoyama T. , Souri M. , Orii K.E. , Kondo N. , Orii T. , Hashimoto T.
      Biochem. Biophys. Res. Commun.217:987-992(1995) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] (ISOFORM 1)
      tissue: Peripheral blood.
    4. 4.
      "Complete sequencing and characterization of 21,243 full-length human cDNAs."
      Ota T. , Suzuki Y. , Nishikawa T. , Otsuki T. , Sugiyama T. , Irie R. , Wakamatsu A. , Hayashi K. , Sato H. , Nagai K. , Kimura K. , Makita H. , Sekine M. , Obayashi M. , Nishi T. , Shibahara T. , Tanaka T. , Ishii S. , more...
      Nat. Genet.36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 3)
      tissue: Cerebellum.
    5. 6.
      "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
      The MGC Project Team
      Genome Res.14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2)
      tissue: Liver.
      tissue: Lung.
      tissue: Pancreas.
    6. 7.
      "Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients."
      Aoyama T. , Souri M. , Ushikubo S. , Kamijo T. , Yamaguchi S. , Kelley R.I. , Rhead W.J. , Uetake K. , Tanaka K. , Hashimoto T.
      J. Clin. Invest.95:2465-2473(1995) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: CHARACTERIZATION
    7. 8.
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: REVIEW ON VARIANTS
    8. 9.
      "Lysine acetylation targets protein complexes and co-regulates major cellular functions."
      Choudhary C. , Kumar C. , Gnad F. , Nielsen M.L. , Rehman M. , Walther T.C. , Olsen J.V. , Mann M.
      Science325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-239 AND LYS-331;IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]
    9. 10.
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]
    10. 11.
      "Structural basis for substrate fatty acyl chain specificity: crystal structure of human very-long-chain acyl-CoA dehydrogenase."
      McAndrew R.P. , Wang Y. , Mohsen A.W. , He M. , Vockley J. , Kim J.J.
      J. Biol. Chem.283:9435-9443(2008) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: X-RAY CRYSTALLOGRAPHY (1.91 ANGSTROMS) OF 69-655 IN COMPLEX WITH THE SUBSTRATE ANALOG MYRISTOYL-COA;FUNCTION;SUBUNIT;COFACTOR;ACTIVE SITE
    11. 12.
      "Mutation analysis of very-long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency: identification and characterization of mutant VLCAD cDNAs from four patients."
      Souri M. , Aoyama T. , Orii K. , Yamaguchi S. , Hashimoto T.
      Am. J. Hum. Genet.58:97-106(1996) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS ACADVLD GLU-130 DEL; LYS-299 DEL; GLN-382 AND TRP-613
    12. 13.
      "Very long chain acyl-coenzyme A dehydrogenase deficiency with adult onset."
      Smelt A.H. , Poorthuis B.J.H.M. , Onkenhout W. , Scholte H.R. , Andresen B.S. , van Duinen S.G. , Gregersen N. , Wintzen A.R.
      Ann. Neurol.43:540-544(1998) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT ACADVLD HIS-450
    13. 14.
      "Molecular heterogeneity in very-long-chain acyl-CoA dehydrogenase deficiency causing pediatric cardiomyopathy and sudden death."
      Mathur A. , Sims H.F. , Gopalakrishnan D. , Gibson B. , Rinaldo P. , Vockley J. , Hug G. , Strauss A.W.
      Circulation99:1337-1343(1999) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS ACADVLD
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