1EGE Electron Transfer date Apr 11, 1996
title Structure Of T255e, E376g Mutant Of Human Medium Chain Acyl-Coa Dehydrogenase
authors H.J.Lee, M.Wang, R.Paschke, A.Nandy, S.Ghisla, J.J.Kim
compound source
Molecule: Medium Chain Acyl-Coa Dehydrogenase
Chain: A, B, C, D
Ec: 1.3.99.3
Biological_unit: Homotetramer
 Organism_scientific: Homo Sapiens
Organism_common: Human
symmetry Space Group: P 43 21 2
R_factor 0.221
crystal
cell
length a length b length c angle alpha angle beta angle gamma
169.590 169.590 151.100 90.00 90.00 90.00
method X-Ray Diffractionresolution 2.75 Å
ligand FADenzyme Acyl-CoA dehydrogenase. Acyl dehydrogenase. Fatty acyl coenzyme A dehydrogenase. Acyl coenzyme A dehydrogenase. Fatty-acyl-CoA dehydrogenase. Acyl CoA dehydrogenase. General acyl CoA dehydrogenase. Medium-chain acyl-coenzyme A dehydrogenase. Medium-chain acyl-CoA dehydrogenase. Long-chain acyl coenzyme A dehydrogenase. Long-chain acyl-CoA dehydrogenase. Oxidoreductase E.C.1.3.99.3
related structures by homologous chain: 1EGC
similarity Belongs to the acyl-coa dehydrogenase family.[Acyl-CoA_dh_M]
subunit Homotetramer.
catalytic activ. Acyl-coa + etf = 2,3-dehydroacyl-coa + reduced etf.
pathway Mitochondrial fatty acid beta-oxidation system; first step.
subcellular loc. Mitochondrial matrix.
gene ACADM (H. sapiens); ACADM (S. scrofa)
function This enzyme is specific for acyl chain lengths of 4 to 16.
disease Acyl-CoA dehydrogenase,medium chain,deficiency of
Defects in acadm are the cause of medium-chain acyl-coa dehydrogenase deficiency (mcad deficiency) [mim. 201450], an autosomal recessive disease which causes fasting hypoglycemia, hepatic dysfunction, and encephalopathy, often resulting in death in infancy. The disease frequency is one in 13000.
Data retrieval
  • Asymmetric unit, PDB entry: [header only] [complete with coordinates] (269 Kb) [Save to disk]
  • LPC: Ligand-Protein Contacts for 1EGE
  • CSU: Contacts of Structural Units for 1EGE
  • Likely Quarternary Molecular Structure file(s) for 1EGE
  • Retrieve 1EGE in mmCIF format [Save to disk]
    • View 1EGE in 3D
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  • RasMol (Install RasMol freeware) Here's help on how to use RasMol.
    • Visual 3D analysis of 1EGE
  • STING, shows the sequence and maps the locations of sequence residues or residue ranges onto the 3D structure.
  • Noncovalent Bond Finder displays the closest contacts to ligand or interface, reports distances, walks over water bridges.
  • GRASS (Graphical Representation and Analysis of Structure Server)
  • Cartoon representation from PDB Cartoon
  • Ramachandran plot from PDBSUm
    • Structure-derived information
  • Dipole moment, from Dipole Server at Weizmann Institute
  • Genome occurence of 1EGE's fold from GeneCensus
  • Classification of representative domains in scop (Structural Classification of Proteins)
        - Domain d1egea2, region A:10-241 [rasmolscript] [script source]
        - Domain d1egea1, region A:242-396 [rasmolscript] [script source]
        - Domain d1egeb2, region B:10-241 [rasmolscript] [script source]
        - Domain d1egeb1, region B:242-396 [rasmolscript] [script source]
        - Domain d1egec2, region C:10-241 [rasmolscript] [script source]
        - Domain d1egec1, region C:242-396 [rasmolscript] [script source]
        - Domain d1eged2, region D:10-241 [rasmolscript] [script source]
        - Domain d1eged1, region D:242-396 [rasmolscript] [script source]
  • Fold representative 1ege from FSSP and Dali (Families of Structurally Similar Proteins)
  • Domain Definition for 1ege from 3Dee ( The Database of Protein Domain Definitions )
  • Class (fold), Architecture (subfold), Topology, Homologous superfamily: CATH
  • Summaries and structural analyses of PDB data files: PDBSum
  • Identification of Protein Pockets & Cavities at CASTp
    • Sequence-derived information
  • View one-letter amino acid or nucleotide sequence for each chain: [1ege_A] [1ege_B] [1ege_C] [1ege_D]
  • SWISS-PROT database: [P11310] [Q9NYF1]
  • Domain organization of [ACDM_HUMAN] by SWISSPFAM
  • Conserved protein region description, domain view and alignment from family Acyl-CoA_dh_M (PF02770) of Pfam.
  • Alignments of the sequence of 1EGE with the sequences similar proteins can be viewed for classification [ACDM_HUMAN] at ProtoMap. Click on "Neighbors List", then on the "See Alignments" button below the list.
  • A sequence distance tree ("phylogenetic tree") can be viewed for 1ACL's classification [ACDM_HUMAN] at ProtoMap. Click on the Cluster number.
    • Other resources with information on 1EGE
  • InterPro: IPR006089 , IPR006090 , IPR006091 , IPR006092
  • PDBREPORT (protein verification by WHAT_CHECK procedures)
  • MMDB (Entrez's Structure Database)
    • Movements, Movies and Images
  • Images from IMB Jena Image Library of Biological Macromolecules.

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