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Gene Review

MOCS1  -  molybdenum cofactor synthesis 1

Homo sapiens

Synonyms: Cell migration-inducing gene 11 protein, MIG11, MOCOD, MOCODA, Molybdenum cofactor biosynthesis protein 1, ...
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Disease relevance of MOCS1


Psychiatry related information on MOCS1

  • In this study, the existence of statistical relationships between intensity difference limens (IDLs) and MOCS functioning was tested in humans [3].

High impact information on MOCS1


Chemical compound and disease context of MOCS1


Biological context of MOCS1

  • MOCS1 and MOCS2 have a bicistronic architecture; i.e., each gene encodes two proteins in different open reading frames [7].
  • The protein products, MOCS1A and B and MOCS2A and B, are expressed either from different mRNAs generated by alternative splicing or by independent translation of a bicistronic mRNA [7].
  • The bicistronic MOCS1 gene has alternative start codons on two mutually exclusive exons [8].
  • DAAM2 gene was linked to the MOCS1 gene on human chromosome 6p21.3 with an interval less than 1 kb [9].
  • We describe here one additional kindred of Arab-Israeli origin, which is also linked to the MOCS1 locus, and demonstrate the feasibility of prenatal diagnosis and carrier detection using microsatellite markers in selected families when mutations are unknown [10].

Anatomical context of MOCS1

  • To investigate the involvement of the medial olivocochlear system (MOCS) in outer hair cell (OHC) motility stabilization, evoked otoacoustic emissions (EOAEs) were recorded in 20 normal-hearing subjects and in eight vestibular-neurotomized subjects, successively in the presence and absence of low-intensity contralateral acoustic stimulation [11].
  • These were: (1) middle ear dysfunction; (2) mild cochlear pathology; (3) central/medial olivocochlear efferent system (MOCS) auditory dysfunction; (4) purely psychological problems; (5) multiple auditory pathologies; (6) combined auditory dysfunction and psychological problems and (7) unknown [12].

Associations of MOCS1 with chemical compounds

  • We showed that for catalytic activity MOCS1A needs an accessible C-terminal double glycine motif [1].
  • Ten novel mutations in the molybdenum cofactor genes MOCS1 and MOCS2 and in vitro characterization of a MOCS2 mutation that abolishes the binding ability of molybdopterin synthase [13].
  • We homozygously identified the CGA insertion after A666 of the MOCS1 gene which produces arginine insertion at codon 222 of MOCS1A [14].
  • The results indicated that anaerobically purified MOCS1A is a monomeric protein containing two oxygen-sensitive FeS clusters, each coordinated by only three cysteine residues [6].
  • MOCS1A shares homology with S-adenosylmethionine (AdoMet)-dependent radical enzymes, which catalyze the formation of protein and/or substrate radicals by reductive cleavage of AdoMet through a [4Fe-4S] cluster [6].

Other interactions of MOCS1


Analytical, diagnostic and therapeutic context of MOCS1

  • MOCS1(-/-) mice could be a suitable animal model for biochemical and/or genetic therapy approaches [16].
  • These observations point to a novel form of microcompartmentalization and render the MOCS genes ideal candidates for a somatic gene therapy approach [17].
  • DESIGN: Transient evoked otoacoustic emissions (TEOAE) and their suppression by the efferent activity of the medial olivocochlear system (MOCS) in response to contralateral acoustic stimulation were used to compare these processes in 18 children with SLI and 21 controls [18].


  1. Functionality of alternative splice forms of the first enzymes involved in human molybdenum cofactor biosynthesis. Hänzelmann, P., Schwarz, G., Mendel, R.R. J. Biol. Chem. (2002) [Pubmed]
  2. New insights into the neuropathogenesis of molybdenum cofactor deficiency. Salman, M.S., Ackerley, C., Senger, C., Becker, L. The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques. (2002) [Pubmed]
  3. Relationship between auditory intensity discrimination in noise and olivocochlear efferent system activity in humans. Micheyl, C., Perrot, X., Collet, L. Behav. Neurosci. (1997) [Pubmed]
  4. Mutations in a polycistronic nuclear gene associated with molybdenum cofactor deficiency. Reiss, J., Cohen, N., Dorche, C., Mandel, H., Mendel, R.R., Stallmeyer, B., Zabot, M.T., Dierks, T. Nat. Genet. (1998) [Pubmed]
  5. A mutation in the gene for the neurotransmitter receptor-clustering protein gephyrin causes a novel form of molybdenum cofactor deficiency. Reiss, J., Gross-Hardt, S., Christensen, E., Schmidt, P., Mendel, R.R., Schwarz, G. Am. J. Hum. Genet. (2001) [Pubmed]
  6. Characterization of MOCS1A, an oxygen-sensitive iron-sulfur protein involved in human molybdenum cofactor biosynthesis. Hänzelmann, P., Hernández, H.L., Menzel, C., García-Serres, R., Huynh, B.H., Johnson, M.K., Mendel, R.R., Schindelin, H. J. Biol. Chem. (2004) [Pubmed]
  7. Mutations in the molybdenum cofactor biosynthetic genes MOCS1, MOCS2, and GEPH. Reiss, J., Johnson, J.L. Hum. Mutat. (2003) [Pubmed]
  8. The bicistronic MOCS1 gene has alternative start codons on two mutually exclusive exons. Gross-Hardt, S., Reiss, J. Mol. Genet. Metab. (2002) [Pubmed]
  9. Identification and characterization of human DAAM2 gene in silico. Katoh, M., Katoh, M. Int. J. Oncol. (2003) [Pubmed]
  10. Prenatal diagnosis and carrier detection for molybdenum cofactor deficiency type A in northern Israel using polymorphic DNA markers. Shalata, A., Mandel, H., Dorche, C., Zabot, M.T., Shalev, S., Hugeirat, Y., Arieh, D., Ronit, Z., Reiss, J., Anbinder, Y., Cohen, N. Prenat. Diagn. (2000) [Pubmed]
  11. Medial olivocochlear system stabilizes active cochlear micromechanical properties in humans. Maison, S., Micheyl, C., Chays, A., Collet, L. Hear. Res. (1997) [Pubmed]
  12. Subcategories of patients with King-Kopetzky syndrome. Zhao, F., Stephens, D. British journal of audiology. (2000) [Pubmed]
  13. Ten novel mutations in the molybdenum cofactor genes MOCS1 and MOCS2 and in vitro characterization of a MOCS2 mutation that abolishes the binding ability of molybdopterin synthase. Leimkühler, S., Charcosset, M., Latour, P., Dorche, C., Kleppe, S., Scaglia, F., Szymczak, I., Schupp, P., Hahnewald, R., Reiss, J. Hum. Genet. (2005) [Pubmed]
  14. A Turkish case with molybdenum cofactor deficiency. Ichida, K., Ibrahim Aydin, H., Hosoyamada, M., Kalkanoglu, H.S., Dursun, A., Ohno, I., Coskun, T., Tokatli, A., Shibasaki, T., Hosoya, T. Nucleosides Nucleotides Nucleic Acids (2006) [Pubmed]
  15. Gephyrin: does splicing affect its function? Paarmann, I., Saiyed, T., Schmitt, B., Betz, H. Biochem. Soc. Trans. (2006) [Pubmed]
  16. Molybdenum cofactor-deficient mice resemble the phenotype of human patients. Lee, H.J., Adham, I.M., Schwarz, G., Kneussel, M., Sass, J.O., Engel, W., Reiss, J. Hum. Mol. Genet. (2002) [Pubmed]
  17. Genetics of molybdenum cofactor deficiency. Reiss, J. Hum. Genet. (2000) [Pubmed]
  18. Contralateral suppression of otoacoustic emissions in children with specific language impairment. Clarke, E.M., Ahmmed, A., Parker, D., Adams, C. Ear and hearing. (2006) [Pubmed]
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