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Gene Review

GMCL1  -  germ cell-less, spermatogenesis associated 1

Homo sapiens

Synonyms: BTBD13, FLJ13057, GCL, GCL1, Germ cell-less protein-like 1, ...
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Disease relevance of GMCL1

  • Ectopic Shh expression in the regenerating retina also results in a decrease in the number of ganglion cells present and an increase in apoptosis mostly in the presumptive ganglion cell layer (GCL) [1].
  • The preference for the GCL could not be explained by the position of the PN graft in the Cb, a selective denervation of the GCL, local damage to other neurons, or the distribution of reactive gliosis in the vicinity of the graft [2].
  • Modifier genes may account for the phenotypic variability observed in the late-onset forms of globoid cell leukodystrophy (GCL) in humans [3].
  • To determine the number of neurons remaining in the ganglion cell layer (GCL) of eyes with nonexudative and exudative age-related macular degeneration (NEAMD and EXAMD, respectively) in relation to photoreceptor loss in the same retinas [4].
  • The thickness of inner plexiform layer (IPL) and inner nuclear layer (INL), and the number of cells in the ganglion cell layer (GCL) decreased after ocular hypertension [5].

Psychiatry related information on GMCL1


High impact information on GMCL1

  • Unlike the other lipases and serine proteases, the catalytic triad of GCL is Ser-His-Glu, with glutamic acid replacing the usual aspartate [7].
  • A model system for studying these programs is the ganglion cell layer (GCL) of the vertebrate retina because of its simple and well-described structure and amenability to experimental manipulations [8].
  • The GCL contains approximately equal numbers of ganglion cells and displaced amacrine cells [8].
  • Roughly half of GCL neurons die during development in amniotes, whereas developmental cell death does not occur in the GCL neurons of anamniotes [8].
  • Independent targeting of the catalytic and modulatory subunits by shRNA caused disruption of GCL as assessed by Northern and Western blotting, enzyme activity, and glutathione concentrations [9].

Chemical compound and disease context of GMCL1

  • By using an antiserum to GABA, we found GABA-immunoreactivity (GABA-IR) to be primarily in amacrine cells lying in the inner nuclear layer (INL) or displaced to the ganglion cell layer (GCL) [10].
  • Less Zn(2+) accumulation in the IPL and INL and less neuronal degeneration in the GCL and INL were found in the retinas treated with Ca(2+)-EDTA or TPEN before ischemia [11].
  • To determine if any of the immunoreactive cells in the GCL were ganglion cells, double labeling experiments were performed using rhodamine latex microspheres ('beads') as retrograde neuronal tracers [12].

Biological context of GMCL1

  • In Drosophila, maternal GCL protein is required to establish the germ lineage during embryonic development [13].
  • In mammals, it is suggested that the GCL function is mainly in spermatogenesis and that it might be related to the ability of mouse GCL to repress transcription [13].
  • Neurogenesis and cell death have been studied extensively in the GCL of various amniotes (rodents, chicks, and monkeys) and anamniotes (fish and frogs), and the two processes highlight developmental differences between the groups [8].
  • Detailed analyses of neuronal and astrocyte cell numbers in the ganglion cell layer (GCL) of whole-mounted peripheral retinas from 16 Alzheimer's disease (AD) and 11 control eyes (11 and 9 cases, respectively) demonstrate extensive neuronal loss throughout the entire retina in AD as compared to control eyes [6].
  • Results suggest that cells are lost from the GCL between weeks 14 and 30 of the gestation period, approximately [14].

Anatomical context of GMCL1

  • GCL co-immunoprecipitates with emerin from HeLa cells [15].
  • Here, we show that, in mammalian cells, GCL is co-localized with LAP2beta to the nuclear envelope [16].
  • In contrast, supplying glutathione precursors to neurons from co-cultured astrocytes did not prevent the apoptotic death triggered by GCL knockdown [9].
  • These results suggest that neural stem cell proliferation begins at the SGZ, and that the cells then migrate into the GCL and differentiate mainly into neuronal cells [17].
  • TUB immunoreactivity was most highly concentrated in the GCL, in the inner and outermost regions of the INL, in the outer plexiform layer (OPL), and in the inner segments of photoreceptor cells [18].

Associations of GMCL1 with chemical compounds

  • Thus, specific and independent disruption of each subunit of GCL in neurons can be said to cause a primary decrease in glutathione that is sufficient to promote neurodegeneration [9].
  • Finally, overexpressing the catalytic (but not modulatory) GCL subunit full-length cDNA increased enzyme activity and glutathione concentrations, yielding neurons more resistant to glutamate- or nitric oxide-mediated apoptosis [9].
  • Moreover, neuronal apoptosis by GCL knockdown was rescued by expressing the corresponding subunit full-length cDNA carrying silent mutations within the shRNA target cDNA sequence and by incubating neurons with gamma-glutamylcysteine or glutathione ethyl ester [9].
  • A few cells in the GCL (0.5-1.5%) were glycine positive [19].
  • Two classes of NOS-expressing neurons, type I and type II, had already been distinguished in the INL [Koistinaho, J. & Sagar, S.M. (1995) In Osborne, N.N. & Chader, G.J. (eds), Progress in Retinal and Eye Research, Vol. 15. Oxford University Press, pp. 69-87] and a third one in the GCL [20].

Other interactions of GMCL1

  • We discovered that these "unascribed" domains of emerin mediate direct binding to a transcriptional repressor, germ cell-less (GCL) [15].
  • BAF, however, competes with GCL for binding to emerin in vitro [21].

Analytical, diagnostic and therapeutic context of GMCL1

  • Immunocytochemistry revealed that labeling of the ganglion cell layer (GCL) by IgG from glaucoma patients (POAG: 13/56, 23.2%; NTG: 6/23, 26%) existed at a significantly higher rate than that by IgG from control subjects (2/60, 3.3%; P < 0.05) [22].
  • RESULTS: With RT-PCR it was found that all five sst mRNAs were expressed in the rabbit retina, with highest levels of sst(1) mRNA. sst(1) immunolabeling was localized to amacrine cells in the proximal inner nuclear layer (INL) of all retinal regions and to displaced amacrine cells in the ganglion cell layer (GCL) of the ventral retina [23].
  • The number of PSA-NCAM positive cells increased in the granule cell layer (GCL) of dentate gyrus (DG) by 1.9 to 2.7-fold at 10 and 20 days after the reperfusion [24].
  • These results show the possibility of retroviral-mediated gene therapy for the treatment of GCL [25].
  • In situ hybridization indicated that mRNA for each receptor is expressed in all retinal cell layers during development, but most intensely in the ganglion cell layer (GCL) [26].


  1. The hedgehog pathway is a modulator of retina regeneration. Spence, J.R., Madhavan, M., Ewing, J.D., Jones, D.K., Lehman, B.M., Del Rio-Tsonis, K. Development (2004) [Pubmed]
  2. Synapse formation and preferential distribution in the granule cell layer by regenerating retinal ganglion cell axons guided to the cerebellum of adult hamsters. Zwimpfer, T.J., Aguayo, A.J., Bray, G.M. J. Neurosci. (1992) [Pubmed]
  3. Delayed clinical and pathological signs in twitcher (globoid cell leukodystrophy) mice on a C57BL/6 x CAST/Ei background. Biswas, S., Biesiada, H., Williams, T.D., LeVine, S.M. Neurobiol. Dis. (2002) [Pubmed]
  4. Preservation of ganglion cell layer neurons in age-related macular degeneration. Medeiros, N.E., Curcio, C.A. Invest. Ophthalmol. Vis. Sci. (2001) [Pubmed]
  5. Presence of calpain-induced proteolysis in retinal degeneration and dysfunction in a rat model of acute ocular hypertension. Oka, T., Tamada, Y., Nakajima, E., Shearer, T.R., Azuma, M. J. Neurosci. Res. (2006) [Pubmed]
  6. Retinal pathology in Alzheimer's disease. II. Regional neuron loss and glial changes in GCL. Blanks, J.C., Schmidt, S.Y., Torigoe, Y., Porrello, K.V., Hinton, D.R., Blanks, R.H. Neurobiol. Aging (1996) [Pubmed]
  7. Ser-His-Glu triad forms the catalytic site of the lipase from Geotrichum candidum. Schrag, J.D., Li, Y.G., Wu, S., Cygler, M. Nature (1991) [Pubmed]
  8. Neurogenesis and cell death in the ganglion cell layer of vertebrate retina. Farah, M.H. Brain Res. Brain Res. Rev. (2006) [Pubmed]
  9. Knockdown of glutamate-cysteine ligase by small hairpin RNA reveals that both catalytic and modulatory subunits are essential for the survival of primary neurons. Diaz-Hernandez, J.I., Almeida, A., Delgado-Esteban, M., Fernandez, E., Bolaños, J.P. J. Biol. Chem. (2005) [Pubmed]
  10. Localization of GABA, glycine, glutamate and tyrosine hydroxylase in the human retina. Crooks, J., Kolb, H. J. Comp. Neurol. (1992) [Pubmed]
  11. Inhibition of cyclooxygenase-2 expression by zinc-chelator in retinal ischemia. Choi, J.S., Kim, K.A., Yoon, Y.J., Fujikado, T., Joo, C.K. Vision Res. (2006) [Pubmed]
  12. Localization of GAD- and GABA-like immunoreactivity in ground squirrel retina: retrograde labeling demonstrates GAD-positive ganglion cells. Lugo-García, N., Blanco, R.E. Brain Res. (1991) [Pubmed]
  13. Reduced human germ cell-less (HGCL) expression in azoospermic men with severe germinal cell impairment. Kleiman, S.E., Yogev, L., Gal-Yam, E.N., Hauser, R., Gamzu, R., Botchan, A., Paz, G., Yavetz, H., Maymon, B.B., Schreiber, L., Barzilai, S., Amariglio, N., Rechavi, G., Simon, A.J. J. Androl. (2003) [Pubmed]
  14. Patterns of cell death in the ganglion cell layer of the human fetal retina. Provis, J.M. J. Comp. Neurol. (1987) [Pubmed]
  15. Transcriptional repressor germ cell-less (GCL) and barrier to autointegration factor (BAF) compete for binding to emerin in vitro. Holaska, J.M., Lee, K.K., Kowalski, A.K., Wilson, K.L. J. Biol. Chem. (2003) [Pubmed]
  16. Nuclear membrane protein LAP2beta mediates transcriptional repression alone and together with its binding partner GCL (germ-cell-less). Nili, E., Cojocaru, G.S., Kalma, Y., Ginsberg, D., Copeland, N.G., Gilbert, D.J., Jenkins, N.A., Berger, R., Shaklai, S., Amariglio, N., Brok-Simoni, F., Simon, A.J., Rechavi, G. J. Cell. Sci. (2001) [Pubmed]
  17. Three steps of neural stem cells development in gerbil dentate gyrus after transient ischemia. Iwai, M., Sato, K., Omori, N., Nagano, I., Manabe, Y., Shoji, M., Abe, K. J. Cereb. Blood Flow Metab. (2002) [Pubmed]
  18. Cell-specific expression of tubby gene family members (tub, Tulp1,2, and 3) in the retina. Ikeda, S., He, W., Ikeda, A., Naggert, J.K., North, M.A., Nishina, P.M. Invest. Ophthalmol. Vis. Sci. (1999) [Pubmed]
  19. Glutamate, GABA, and glycine in the human retina: an immunocytochemical investigation. Davanger, S., Ottersen, O.P., Storm-Mathisen, J. J. Comp. Neurol. (1991) [Pubmed]
  20. Effects of brain-derived neurotrophic factor on the development of NADPH-diaphorase/nitric oxide synthase-positive amacrine cells in the rodent retina. Cellerino, A., Arango-González, B.A., Kohler, K. Eur. J. Neurosci. (1999) [Pubmed]
  21. Nuclear membrane protein emerin: roles in gene regulation, actin dynamics and human disease. Wilson, K.L., Holaska, J.M., de Oca, R.M., Tifft, K., Zastrow, M., Segura-Totten, M., Mansharamani, M., Bengtsson, L. Novartis Found. Symp. (2005) [Pubmed]
  22. Retinal ganglion cells recognized by serum autoantibody against gamma-enolase found in glaucoma patients. Maruyama, I., Ohguro, H., Ikeda, Y. Invest. Ophthalmol. Vis. Sci. (2000) [Pubmed]
  23. Expression of somatostatin subtype 1 receptor in the rabbit retina. Cristiani, R., Fontanesi, G., Casini, G., Petrucci, C., Viollet, C., Bagnoli, P. Invest. Ophthalmol. Vis. Sci. (2000) [Pubmed]
  24. Induction of highly polysialylated neural cell adhesion molecule (PSA-NCAM) in postischemic gerbil hippocampus mainly dissociated with neural stem cell proliferation. Iwai, M., Hayashi, T., Zhang, W.R., Sato, K., Manabe, Y., Abe, K. Brain Res. (2001) [Pubmed]
  25. Correction of the galactocerebrosidase deficiency in globoid cell leukodystrophy-cultured cells by SL3-3 retroviral-mediated gene transfer. Gama Sosa, M.A., de Gasperi, R., Undevia, S., Yeretsian, J., Rouse, S.C., Lyerla, T.A., Kolodny, E.H. Biochem. Biophys. Res. Commun. (1996) [Pubmed]
  26. Differential distribution of fibroblast growth factor receptors (FGFRs) on foveal cones: FGFR-4 is an early marker of cone photoreceptors. Cornish, E.E., Natoli, R.C., Hendrickson, A., Provis, J.M. Mol. Vis. (2004) [Pubmed]
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