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Gene Review

LGSN  -  lengsin, lens protein with glutamine...

Homo sapiens

Synonyms: GLULD1, Glutamate-ammonia ligase domain-containing protein 1, LGS, Lengsin, Lens glutamine synthase-like
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Disease relevance of GLULD1

  • Single fiber electromyography ( SFEMG ) of the extensor digitorum communis muscle was performed on 20 patients with either myopathic limb girdle syndromes ( LGS ) or chronic spinal muscular atrophy ( CSMA ) to assess its value in the differential diagnosis of these disorders [1].
  • We find that LGS has six binding sites for the hydrophobic surface probe bis-ANS and relieves cellular toxicity caused by amyloid-beta expression in a folding-impaired yeast mutant [2].
  • STUDY OBJECTIVE: The purpose of this study was to assess the extent of immune dysfunction in a well-defined group of epileptic patients: children with diagnosis of West syndrome (WS) or with transitions to another age-related EEG patterns, the multifocal independent spikes (MIS), and the slow spike-wave complexes (Lennox-Gastaut syndrome - LGS) [3].

High impact information on GLULD1

  • While documenting the structural similarity between LGS and prokaryotic GS-I, the data rule out any involvement of lengsin in glutamine biosynthesis and suggest an unrelated role that may be important for lens homeostasis and transparency [2].
  • The discovery of an abundant, novel transcript, lengsin, and a major novel splice form of MP19 reflect the utility of unamplified libraries constructed from dissected tissue [4].
  • Among the most abundant cDNAs is one for a novel gene related to glutamine synthetase, which was designated "lengsin" (LGS) [4].
  • Lengsin Is a Survivor of an Ancient Family of Class I Glutamine Synthetases Re-engineered by Evolution for a Role in the Vertebrate Lens [5].
  • Lengsin is a major protein of the vertebrate eye lens [5].

Biological context of GLULD1

  • METHOD: A group of 50 patients (33 with WS, 10 with LGS and 7 with MIS) and 20 age-matched healthy controls were submitted to enumeration of T lymphocyte subsets: CD1, CD3, CD4, CD8, CD4/CD8 ratio and lymphocyte proliferation assay to phytohaemagglutinin (PHA), in the presence of autologous and AB, homologous plasma [3].

Associations of GLULD1 with chemical compounds


Other interactions of GLULD1

  • ELOVL5, SMAP1 and GLULD1 were selected on the basis of their location, tissue expression and/or function [6].

Analytical, diagnostic and therapeutic context of GLULD1


  1. Single fiber electromyography in the differential diagnosis of myopathic limb girdle syndromes and chronic spinal muscular atrophy. Shields, R.W. Muscle Nerve (1984) [Pubmed]
  2. Structural and functional properties of lengsin, a pseudo-glutamine synthetase in the transparent human lens. Grassi, F., Moretto, N., Rivetti, C., Cellai, S., Betti, M., M??rquez, A.J., Maraini, G., Ottonello, S. Biochem. Biophys. Res. Commun. (2006) [Pubmed]
  3. Immunologic aspects of West syndrome and evidence of plasma inhibitory effects on T cell function. Montelli, T.C., Soares, A.M., Peraçoli, M.T. Arquivos de neuro-psiquiatria. (2003) [Pubmed]
  4. Expressed sequence tag analysis of adult human lens for the NEIBank Project: over 2000 non-redundant transcripts, novel genes and splice variants. Wistow, G., Bernstein, S.L., Wyatt, M.K., Behal, A., Touchman, J.W., Bouffard, G., Smith, D., Peterson, K. Mol. Vis. (2002) [Pubmed]
  5. Lengsin Is a Survivor of an Ancient Family of Class I Glutamine Synthetases Re-engineered by Evolution for a Role in the Vertebrate Lens. Wyatt, K., White, H.E., Wang, L., Bateman, O.A., Slingsby, C., Orlova, E.V., Wistow, G. Structure (2006) [Pubmed]
  6. Mutation screening of three candidate genes, ELOVL5, SMAP1 and GLULD1 in autosomal recessive retinitis pigmentosa. Barragan, I., Marcos, I., Borrego, S., Antiñolo, G. Int. J. Mol. Med. (2005) [Pubmed]
  7. Sensitivity studies of AutoPap System Location-Guided Screening of cervical-vaginal cytologic smears. Huang, T.W., Lin, T.S., Lee, J.S. Acta Cytol. (1999) [Pubmed]
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