Gene Symbol: Uchl1
Description: ubiquitin carboxy-terminal hydrolase L1
Alias: AW822034, C88048, PGP 9.5, PGP9.5, R75593, UCH-L1, UCHL-1, gad, ubiquitin carboxyl-terminal hydrolase isozyme L1, gracile axonal dystrophy, neuron cytoplasmic protein 9.5, protein gene product 9.5, ubiquitin thioesterase L1
Species: mouse
Products:     Uchl1

Top Publications

  1. Yamazaki K, Wakasugi N, Tomita T, Kikuchi T, Mukoyama M, Ando K. Gracile axonal dystrophy (GAD), a new neurological mutant in the mouse. Proc Soc Exp Biol Med. 1988;187:209-15 pubmed
    ..The mutation is inherited as an autosomal recessive trait. It was, therefore, named gracile axonal dystrophy (GAD) with the gene symbol gad...
  2. Kabuta T, Furuta A, Aoki S, Furuta K, Wada K. Aberrant interaction between Parkinson disease-associated mutant UCH-L1 and the lysosomal receptor for chaperone-mediated autophagy. J Biol Chem. 2008;283:23731-8 pubmed publisher
  3. Yasuda T, Nihira T, Ren Y, Cao X, Wada K, Setsuie R, et al. Effects of UCH-L1 on alpha-synuclein over-expression mouse model of Parkinson's disease. J Neurochem. 2009;108:932-44 pubmed publisher
    ..vulnerabilities of dopaminergic (DA) neurons in UCH-L1-transgenic (Tg) and spontaneous UCH-L1-null gracile axonal dystrophy mice to an important PD-causing insult, abnormal accumulation of alpha-synuclein (alphaSyn)...
  4. Zhong J, Li X, McNamee C, Chen A, Baccarini M, Snider W. Raf kinase signaling functions in sensory neuron differentiation and axon growth in vivo. Nat Neurosci. 2007;10:598-607 pubmed
    ..We conclude that Raf function is crucial for several aspects of DRG neuron development, including differentiation and axon growth. ..
  5. Okubo T, Pevny L, Hogan B. Sox2 is required for development of taste bud sensory cells. Genes Dev. 2006;20:2654-9 pubmed
  6. Gascon E, Gaillard S, Malapert P, Liu Y, Rodat Despoix L, Samokhvalov I, et al. Hepatocyte growth factor-Met signaling is required for Runx1 extinction and peptidergic differentiation in primary nociceptive neurons. J Neurosci. 2010;30:12414-23 pubmed publisher
    ..Together, our novel findings support a model in which peptidergic versus nonpeptidergic specification depends on a balance between HGF-Met signaling and Runx1 extinction/maintenance. ..
  7. Suzuki Y, Ikeda K, Kawakami K. Development of gustatory papillae in the absence of Six1 and Six4. J Anat. 2011;219:710-21 pubmed publisher
    ..The foliate papillae elevated initially, and showed stunted trenches. Therefore, Six1 and Six4 function synergistically to form gustatory papillae during development of the tongue. ..
  8. Kurihara L, Kikuchi T, Wada K, Tilghman S. Loss of Uch-L1 and Uch-L3 leads to neurodegeneration, posterior paralysis and dysphagia. Hum Mol Genet. 2001;10:1963-70 pubmed
    ..For example, gracile axonal dystrophy (gad) mutant mice, which harbor a deletion within the gene encoding ubiquitin C-terminal hydrolase L1 (..
  9. Chen D, Jarrell A, Guo C, Lang R, Atit R. Dermal ?-catenin activity in response to epidermal Wnt ligands is required for fibroblast proliferation and hair follicle initiation. Development. 2012;139:1522-33 pubmed publisher
    ..These data reveal functional roles for dermal Wnt signaling/?-catenin in fibroblast proliferation and in the epidermal hair follicle initiation program. ..

More Information


  1. Ichihara N, Wu J, Chui D, Yamazaki K, Wakabayashi T, Kikuchi T. Axonal degeneration promotes abnormal accumulation of amyloid beta-protein in ascending gracile tract of gracile axonal dystrophy (GAD) mouse. Brain Res. 1995;695:173-8 pubmed
    The GAD mouse is a spontaneous neurological mutant with axonal dystrophy in the gracile tract of the medulla oblongata and spinal cord...
  2. Zhang H, Sun Y, Hu R, Luo W, Mao X, Zhao Z, et al. The regulation of the UCH-L1 gene by transcription factor NF-?B in podocytes. Cell Signal. 2013;25:1574-85 pubmed publisher
  3. Hall J, Hooper J, Finger T. Expression of sonic hedgehog, patched, and Gli1 in developing taste papillae of the mouse. J Comp Neurol. 1999;406:143-55 pubmed
  4. Chen F, Sugiura Y, Myers K, Liu Y, Lin W. Ubiquitin carboxyl-terminal hydrolase L1 is required for maintaining the structure and function of the neuromuscular junction. Proc Natl Acad Sci U S A. 2010;107:1636-41 pubmed publisher
    ..These findings demonstrate that UCH-L1 is required for the maintenance of the structure and function of the NMJ and that the loss of normal UCH-L1 activity may result in neurodegeneration in the peripheral nervous system. ..
  5. Cartier A, Djakovic S, Salehi A, Wilson S, Masliah E, Patrick G. Regulation of synaptic structure by ubiquitin C-terminal hydrolase L1. J Neurosci. 2009;29:7857-68 pubmed publisher
    ..These findings point to a significant role of UCH-L1 in synaptic remodeling, most likely by modulating free monomeric ubiquitin levels in an activity-dependent manner. ..
  6. Hall J, Bell M, Finger T. Disruption of sonic hedgehog signaling alters growth and patterning of lingual taste papillae. Dev Biol. 2003;255:263-77 pubmed
  7. Kabuta T, Mitsui T, Takahashi M, Fujiwara Y, Kabuta C, Konya C, et al. Ubiquitin C-terminal hydrolase L1 (UCH-L1) acts as a novel potentiator of cyclin-dependent kinases to enhance cell proliferation independently of its hydrolase activity. J Biol Chem. 2013;288:12615-26 pubmed publisher
    ..We concluded that UCH-L1 is a novel regulator of the kinase activities of CDKs. We believe that our findings from this study will significantly contribute to our understanding of cell cycle-associated diseases. ..
  8. Young H, Bergner A, Muller T. Acquisition of neuronal and glial markers by neural crest-derived cells in the mouse intestine. J Comp Neurol. 2003;456:1-11 pubmed
    ..5(+) nor S100b(+). Because some classes of neurons are not present in significant numbers until after birth, the expression of PGP9.5 by developing enteric neurons appeared to precede the expression of neuron type-specific markers. ..
  9. Setsuie R, Wang Y, Mochizuki H, Osaka H, Hayakawa H, Ichihara N, et al. Dopaminergic neuronal loss in transgenic mice expressing the Parkinson's disease-associated UCH-L1 I93M mutant. Neurochem Int. 2007;50:119-29 pubmed
    ..We generated transgenic (Tg) mice carrying human UCHL1 under control of the PDGF-B promoter; two independent lines were generated with the I93M mutation (a high- and low-..
  10. Beites C, Hollenbeck P, Kim J, Lovell Badge R, Lander A, Calof A. Follistatin modulates a BMP autoregulatory loop to control the size and patterning of sensory domains in the developing tongue. Development. 2009;136:2187-97 pubmed publisher
  11. Walters B, Campbell S, Chen P, Taylor A, Schroeder D, Dobrunz L, et al. Differential effects of Usp14 and Uch-L1 on the ubiquitin proteasome system and synaptic activity. Mol Cell Neurosci. 2008;39:539-48 pubmed publisher
  12. Hussain S, Feldman A, Das C, Ziesmer S, Ansell S, Galardy P. Ubiquitin hydrolase UCH-L1 destabilizes mTOR complex 1 by antagonizing DDB1-CUL4-mediated ubiquitination of raptor. Mol Cell Biol. 2013;33:1188-97 pubmed publisher
    ..These events lead to mTORC1 dissolution and a secondary increase in mTORC2. Experiments in Uchl1-deficient and transgenic mice suggest that the balance between these pathways is important for preventing ..
  13. Luo W, Wickramasinghe S, Savitt J, Griffin J, Dawson T, Ginty D. A hierarchical NGF signaling cascade controls Ret-dependent and Ret-independent events during development of nonpeptidergic DRG neurons. Neuron. 2007;54:739-54 pubmed
    ..These findings support a model in which NGF controls maturation of nonpeptidergic DRG neurons through a combination of GFR/Ret-dependent and -independent signaling pathways. ..
  14. Nakayama A, Miura H, Shindo Y, Kusakabe Y, Tomonari H, Harada S. Expression of the basal cell markers of taste buds in the anterior tongue and soft palate of the mouse embryo. J Comp Neurol. 2008;509:211-24 pubmed publisher
    ..This is the first report describing the time courses of the embryonic expression of basal cell markers of taste buds...
  15. Bheda A, Yue W, Gullapalli A, Whitehurst C, Liu R, Pagano J, et al. Positive reciprocal regulation of ubiquitin C-terminal hydrolase L1 and beta-catenin/TCF signaling. PLoS ONE. 2009;4:e5955 pubmed publisher
    ..Ubiquitin C-terminal Hydrolase L1 (UCH L1) has been linked to several neurological diseases as well as human cancer, but the physiological targets and the ..
  16. Saigoh K, Wang Y, Suh J, Yamanishi T, Sakai Y, Kiyosawa H, et al. Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice. Nat Genet. 1999;23:47-51 pubmed
    The gracile axonal dystrophy (gad) mouse is an autosomal recessive mutant that shows sensory ataxia at an early stage, followed by motor ataxia at a later stage...
  17. Wang Y, Takeda A, Osaka H, Hara Y, Furuta A, Setsuie R, et al. Accumulation of beta- and gamma-synucleins in the ubiquitin carboxyl-terminal hydrolase L1-deficient gad mouse. Brain Res. 2004;1019:1-9 pubmed
    ..to demonstrate accelerated accumulation of beta- and gamma-synucleins in axonal spheroids in gracile axonal dystrophy (gad) mice, which do not express ubiquitin carboxyl-terminal hydrolase L1 (UCH-L1)...
  18. Osaka H, Wang Y, Takada K, Takizawa S, Setsuie R, Li H, et al. Ubiquitin carboxy-terminal hydrolase L1 binds to and stabilizes monoubiquitin in neuron. Hum Mol Genet. 2003;12:1945-58 pubmed
    ..Mutations in UCH L1 are linked to Parkinson's disease as well as gracile axonal dystrophy (gad) in mice...
  19. Wakisaka S, Miyawaki Y, Youn S, Kato J, Kurisu K. Protein gene-product 9.5 in developing mouse circumvallate papilla: comparison with neuron-specific enolase and calcitonin gene-related peptide. Anat Embryol (Berl). 1996;194:365-72 pubmed
    ..5-IR nerve fibers. The present results indicate that invasion by nerve fibers of the epithelium of lingual papillae occurs in a complex manner, and that these nerve fibers may participate in the formation of the taste buds. ..
  20. Ito T, Udaka N, Yazawa T, Okudela K, Hayashi H, Sudo T, et al. Basic helix-loop-helix transcription factors regulate the neuroendocrine differentiation of fetal mouse pulmonary epithelium. Development. 2000;127:3913-21 pubmed
    ..Thus, the neuroendocrine differentiation depends on basic helix-loop-helix factors, and Notch/Notch-ligand pathways may be involved in determining the cell differentiation fate in fetal airway epithelium. ..
  21. Vaziri Sani F, Kaartinen V, El Shahawy M, Linde A, Gritli Linde A. Developmental changes in cellular and extracellular structural macromolecules in the secondary palate and in the nasal cavity of the mouse. Eur J Oral Sci. 2010;118:221-36 pubmed publisher
    ..The hitherto-unknown innervation pattern of the developing palate was revealed. These findings may be of value for unravelling the pathogenesis of palatal clefting. ..
  22. Hasegawa H, Abbott S, Han B, Qi Y, Wang F. Analyzing somatosensory axon projections with the sensory neuron-specific Advillin gene. J Neurosci. 2007;27:14404-14 pubmed
    ..Functionally, we found that the regenerative axon growth of Advillin-null sensory neurons is significantly shortened and that deletion of Advillin reduces the plasticity of whisker-related barrelettes patterns in the hindbrain. ..
  23. Kist R, Watson M, Crosier M, Robinson M, Fuchs J, Reichelt J, et al. The formation of endoderm-derived taste sensory organs requires a Pax9-dependent expansion of embryonic taste bud progenitor cells. PLoS Genet. 2014;10:e1004709 pubmed publisher
    ..In this pathway, Pax9 is essential to generate a pool of taste bud progenitors and to maintain their competence towards prosensory cell fate induction. ..
  24. O Donovan K, Ma K, Guo H, Wang C, Sun F, Han S, et al. B-RAF kinase drives developmental axon growth and promotes axon regeneration in the injured mature CNS. J Exp Med. 2014;211:801-14 pubmed publisher
    ..We conclude that cell-intrinsic RAF signaling is a crucial pathway promoting developmental and regenerative axon growth in the peripheral and central nervous systems. ..
  25. Sekiguchi S, Kwon J, Yoshida E, Hamasaki H, Ichinose S, Hideshima M, et al. Localization of ubiquitin C-terminal hydrolase L1 in mouse ova and its function in the plasma membrane to block polyspermy. Am J Pathol. 2006;169:1722-9 pubmed
    ..the functional role of UCH-L1 in mouse eggs, we analyzed the fertilization rate of UCH-L1-deficient ova of gad female mice...
  26. Le Moine C, Fauchey V, Jaber M. Opioid receptor gene expression in dopamine transporter knock-out mice in adult and during development. Neuroscience. 2002;112:131-9 pubmed
  27. Ieda M, Fukuda K, Hisaka Y, Kimura K, Kawaguchi H, Fujita J, et al. Endothelin-1 regulates cardiac sympathetic innervation in the rodent heart by controlling nerve growth factor expression. J Clin Invest. 2004;113:876-84 pubmed
    ..These findings indicate that endothelin-1 regulates NGF expression in cardiomyocytes and plays a critical role in sympathetic innervation of the heart. ..
  28. Oda K, Yamazaki K, Miura H, Shibasaki H, Kikuchi T. Dying back type axonal degeneration of sensory nerve terminals in muscle spindles of the gracile axonal dystrophy (GAD) mutant mouse. Neuropathol Appl Neurobiol. 1992;18:265-81 pubmed
    A disorder of the gracile axonal dystrophy (GAD) mutant mouse is characterized by a neuromuscular disease with sensory ataxia (detectable 30 days after birth) and paresis of the hindlimbs (detectable at 80 days)...
  29. Bates M, Dunagan D, Welch L, Kaul A, Harvey R. The Hlx homeobox transcription factor is required early in enteric nervous system development. BMC Dev Biol. 2006;6:33 pubmed
    ..5-16.5. The Hlx homeobox transcription factor is required for early aspects of ENS development. ..
  30. Lombardino A, Li X, Hertel M, Nottebohm F. Replaceable neurons and neurodegenerative disease share depressed UCHL1 levels. Proc Natl Acad Sci U S A. 2005;102:8036-41 pubmed publisher
    ..showed a consistent and robust underexpression of the deubiquitination gene ubiquitin carboxyl-terminal hydrolase (UCHL1) that is involved with protein degradation...
  31. Gong S. Characterization of olfactory nerve abnormalities in Twirler mice. Differentiation. 2001;69:58-65 pubmed
    ..The defects in the Twirler may prove to be a valuable system to analyze problems in olfactory pathfinding and maturation. ..
  32. Jiang Y, Liu M, Gershon M. Netrins and DCC in the guidance of migrating neural crest-derived cells in the developing bowel and pancreas. Dev Biol. 2003;258:364-84 pubmed
    ..Netrins also promoted the survival/development of enteric crest-derived cells. The formation of submucosal and pancreatic ganglia thus involves the attraction of DCC-expressing crest-derived cells by netrins. ..
  33. Owens S, Broman K, Wiltshire T, Elmore J, Bradley K, Smith J, et al. Genome-wide linkage identifies novel modifier loci of aganglionosis in the Sox10Dom model of Hirschsprung disease. Hum Mol Genet. 2005;14:1549-58 pubmed
  34. Hasegawa H, Wang F. Visualizing mechanosensory endings of TrkC-expressing neurons in HS3ST-2-hPLAP mice. J Comp Neurol. 2008;511:543-56 pubmed publisher
    ..Our results revealed the subtype identities of TrkC-positive mechanosensory neurons and demonstrated the usefulness of HS3ST-2 as a genetic marker for these subclasses of neurons. ..
  35. Chirivella L, Cano Jaimez M, Pérez Sánchez F, Herraez L, Carretero J, Farinas I, et al. IRS2 signalling is required for the development of a subset of sensory spinal neurons. Eur J Neurosci. 2012;35:341-52 pubmed publisher
    ..Collectively, these findings indicate that IRS2 signalling is required for the proper development of spinal sensory neurons involved in the perception of pain. ..
  36. Teng L, Mundell N, Frist A, Wang Q, Labosky P. Requirement for Foxd3 in the maintenance of neural crest progenitors. Development. 2008;135:1615-24 pubmed publisher
  37. Mu X, Fu X, Beremand P, Thomas T, Klein W. Gene regulation logic in retinal ganglion cell development: Isl1 defines a critical branch distinct from but overlapping with Pou4f2. Proc Natl Acad Sci U S A. 2008;105:6942-7 pubmed publisher
    ..They also reveal that identical RGC expression patterns are achieved by different combinations of divergent inputs from upstream transcription factors. ..
  38. Mtango N, Sutovsky M, Susor A, Zhong Z, Latham K, Sutovsky P. Essential role of maternal UCHL1 and UCHL3 in fertilization and preimplantation embryo development. J Cell Physiol. 2012;227:1592-603 pubmed publisher
    ..This study examined the roles of UCHL1 and UCHL3, two members of ubiquitin C-terminal hydrolase (UCH) family of DUBs, in murine fertilization and ..
  39. Tsao P, Vasconcelos M, Izvolsky K, Qian J, LU J, Cardoso W. Notch signaling controls the balance of ciliated and secretory cell fates in developing airways. Development. 2009;136:2297-307 pubmed publisher
    ..It might also be relevant to mediate the metaplastic changes in the respiratory epithelium that occur in pathological conditions, such as asthma and chronic obstructive pulmonary disease. ..
  40. Pfeffer M, Plenzig S, Gispert S, Wada K, Korf H, Von Gall C. Disturbed sleep/wake rhythms and neuronal cell loss in lateral hypothalamus and retina of mice with a spontaneous deletion in the ubiquitin carboxyl-terminal hydrolase L1 gene. Neurobiol Aging. 2012;33:393-403 pubmed publisher
    ..rhythms, orexin A-immunoreaction (Ir) in the lateral hypothalamus (LH) and melanopsin-Ir in the retina of gracile axonal dystrophy (gad) mice with a spontaneous deletion in the Uch-l1 gene...
  41. Yamazaki K, Oda K, Endo C, Kikuchi T, Wakabayashi T. Methylcobalamin (methyl-B12) promotes regeneration of motor nerve terminals degenerating in anterior gracile muscle of gracile axonal dystrophy (GAD) mutant mouse. Neurosci Lett. 1994;170:195-7 pubmed
    ..methyl-B12, mecobalamin) on degeneration of motor nerve terminals in the anterior gracile muscle of gracile axonal dystrophy (GAD) mutant mice...
  42. Lähteenmäki M, Kupari J, Airaksinen M. Increased apoptosis of parasympathetic but not enteric neurons in mice lacking GFRalpha2. Dev Biol. 2007;305:325-32 pubmed
  43. Kwon J, Mochida K, Wang Y, Sekiguchi S, Sankai T, Aoki S, et al. Ubiquitin C-terminal hydrolase L-1 is essential for the early apoptotic wave of germinal cells and for sperm quality control during spermatogenesis. Biol Reprod. 2005;73:29-35 pubmed
    ..Previously, we found that UCHL1-deficient testes of gad mice have reduced ubiquitin levels and are resistant to cryptorchid stress-related injury...
  44. Guo Y, Lu Y, Zheng Y, Chen X, Dong J, Yuan R, et al. Ubiquitin C-Terminal Hydrolase L1 (UCH-L1) Promotes Hippocampus-Dependent Memory via Its Deubiquitinating Effect on TrkB. J Neurosci. 2017;37:5978-5995 pubmed publisher
    ..Overall, our study provides novel insights into the mechanisms of UCH-L1-mediated neurobiological functions and suggests that ubiquitination is an important regulatory signal for TrkB functions. ..
  45. Li C, Li A, Xing Y, Li M, Chan B, Ouyang R, et al. Apc deficiency alters pulmonary epithelial cell fate and inhibits Nkx2.1 via triggering TGF-beta signaling. Dev Biol. 2013;378:13-24 pubmed publisher
    ..deficiency altered lung epithelial cell fate by inhibiting Clara and ciliated cell differentiation and activating Uchl1, a marker of neuroendocrine cells. Similar to PNEC in normal lung, Uchl1(positive) cells were innervated...
  46. Packard A, Schnittke N, Romano R, Sinha S, Schwob J. DeltaNp63 regulates stem cell dynamics in the mammalian olfactory epithelium. J Neurosci. 2011;31:8748-59 pubmed publisher
    ..Together, our data identify a novel mechanism for the generation of a reserve stem cell population and suggest that a p63-dependent molecular switch is responsible for activating reserve stem cells when they are needed. ..
  47. Zhang X, Guo L, Niu T, Shao L, Li H, Wu W, et al. Ubiquitin carboxyl terminal hydrolyase L1-suppressed autophagic degradation of p21WAF1/Cip1 as a novel feedback mechanism in the control of cardiac fibroblast proliferation. PLoS ONE. 2014;9:e94658 pubmed publisher
  48. Xing Y, Li C, Li A, Sridurongrit S, Tiozzo C, Bellusci S, et al. Signaling via Alk5 controls the ontogeny of lung Clara cells. Development. 2010;137:825-33 pubmed publisher
    ..Thus, the signaling pathway by which TGFbeta/ALK5 regulates Clara cell differentiation may entail inhibition of Pten expression, which in turn activates ERK and AKT phosphorylation. ..
  49. Suh J, Yamazaki A, Tomita T. Breeding of the gad-mdx mouse: influence of genetically induced denervation on dystrophic muscle fibers. Lab Anim Sci. 1994;44:42-6 pubmed
    A new double mutant mouse strain, gad-mdx, was established...
  50. Jia S, Wildner H, Birchmeier C. Insm1 controls the differentiation of pulmonary neuroendocrine cells by repressing Hes1. Dev Biol. 2015;408:90-8 pubmed publisher
    ..Our work demonstrates that Insm1 is a key factor regulating differentiation of pulmonary neuroendocrine cells. ..
  51. Finelli M, Murphy K, Chen L, Zou H. Differential phosphorylation of Smad1 integrates BMP and neurotrophin pathways through Erk/Dusp in axon development. Cell Rep. 2013;3:1592-606 pubmed publisher
    ..Together, the BMP and neurotrophin pathways form a tightly regulated signaling network with a balanced ratio of Erk1/2 and pErk1/2 to direct the precise connections between sensory neurons and peripheral targets. ..
  52. Miura H, Oda K, Endo C, Yamazaki K, Shibasaki H, Kikuchi T. Progressive degeneration of motor nerve terminals in GAD mutant mouse with hereditary sensory axonopathy. Neuropathol Appl Neurobiol. 1993;19:41-51 pubmed
    The evolution of motor nerve degeneration was examined in gracile axonal dystrophy (GAD) mutant mice, which develop initial sensory ataxia and subsequent motor paresis...
  53. Kon Y, Endoh D, Iwanaga T. Expression of protein gene product 9.5, a neuronal ubiquitin C-terminal hydrolase, and its developing change in sertoli cells of mouse testis. Mol Reprod Dev. 1999;54:333-41 pubmed
    ..5 in mice was highly conserved, like that in other mammals. These results suggest that PGP9.5 is a useful marker for activated Sertoli cells, playing an important role in degradation of abnormal proteins. ..
  54. Gou Fàbregas M, Macià A, Anerillas C, Vaquero M, Jové M, Jain S, et al. 7-dehydrocholesterol efficiently supports Ret signaling in a mouse model of Smith-Opitz-Lemli syndrome. Sci Rep. 2016;6:28534 pubmed publisher
    ..Finally, replacement experiments demonstrated that 7-DHC efficiently supports Ret signaling in vitro. Taken together, our findings do not support a role of Ret signaling in the pathogenesis of SLOS. ..
  55. Scott A, Hasegawa H, Sakurai K, Yaron A, Cobb J, Wang F. Transcription factor short stature homeobox 2 is required for proper development of tropomyosin-related kinase B-expressing mechanosensory neurons. J Neurosci. 2011;31:6741-9 pubmed publisher
  56. Read N, Gutsol A, Holterman C, Carter A, Coulombe J, Gray D, et al. Ubiquitin C-terminal hydrolase L1 deletion ameliorates glomerular injury in mice with ACTN4-associated focal segmental glomerulosclerosis. Biochim Biophys Acta. 2014;1842:1028-40 pubmed publisher
    Renal ubiquitin C-terminal hydrolase L1 (UCHL1) is upregulated in a subset of human glomerulopathies, including focal segmental glomerulosclerosis (FSGS), where it may serve to promote ubiquitin pools for degradation of cytotoxic ..
  57. Nunes A, Liz M, Franquinho F, Teixeira L, Sousa V, Chenu C, et al. Neuropeptide Y expression and function during osteoblast differentiation--insights from transthyretin knockout mice. FEBS J. 2010;277:263-75 pubmed publisher
  58. Zhu S, Barbe M, Amin N, Rani S, Popoff S, Safadi F, et al. Immunolocalization of Periostin-like factor and Periostin during embryogenesis. J Histochem Cytochem. 2008;56:329-45 pubmed
    ..The early expression of PLF in brain differentiation zones and in developing axon bundles and nerves suggests that it may facilitate axon growth. ..
  59. Cachaço A, Chuva de Sousa Lopes S, Kuikman I, Bajanca F, Abe K, Baudoin C, et al. Knock-in of integrin beta 1D affects primary but not secondary myogenesis in mice. Development. 2003;130:1659-71 pubmed
    ..Together these results demonstrate for the first time distinct roles for beta1 integrins in primary versus secondary myogenesis and that the beta 1A and beta 1D variants are not functionally equivalent in this process. ..
  60. Kameda Y, Nishimaki T, Chisaka O, Iseki S, Sucov H. Expression of the epithelial marker E-cadherin by thyroid C cells and their precursors during murine development. J Histochem Cytochem. 2007;55:1075-88 pubmed
    ..These results indicated that murine thyroid C cells are derived from the endodermal epithelial cells of the fourth pharyngeal pouch and do not originate from neural crest cells. ..
  61. Kabuta T, Setsuie R, Mitsui T, Kinugawa A, Sakurai M, Aoki S, et al. Aberrant molecular properties shared by familial Parkinson's disease-associated mutant UCH-L1 and carbonyl-modified UCH-L1. Hum Mol Genet. 2008;17:1482-96 pubmed publisher
    ..Our results also suggest that the carbonyl modification of UCH-L1 and subsequent abnormal interactions of carbonyl-modified UCH-L1 with multiple proteins, including tubulin, constitute one of the causes of sporadic PD. ..
  62. Gu Y, Chen Q, Gu Z, Shi Y, Yao Y, Wang J, et al. Ubiquitin carboxyl-terminal hydrolase L1 contributes to the oocyte selective elimination in prepubertal mouse ovaries. Sheng Li Xue Bao. 2009;61:175-84 pubmed
    ..These results suggest that UCH-L1 plays an important role, possibly in association with Jab1 and p27(Kipl), in selective elimination of abnormal oocytes during mouse prepubertal development. ..
  63. Nekrep N, Wang J, Miyatsuka T, German M. Signals from the neural crest regulate beta-cell mass in the pancreas. Development. 2008;135:2151-60 pubmed publisher
    ..2 form a non-cell-autonomous feedback loop that links the neural crest with the pancreatic epithelium, regulates the size of the beta-cell population, and thereby impacts insulin-secretory capacity and energy homeostasis. ..
  64. Poon W, Carlos A, Aguilar B, Berchtold N, Kawano C, Zograbyan V, et al. ?-Amyloid (A?) oligomers impair brain-derived neurotrophic factor retrograde trafficking by down-regulating ubiquitin C-terminal hydrolase, UCH-L1. J Biol Chem. 2013;288:16937-48 pubmed publisher
    ..Further, our results support the idea that in AD, A? may down-regulate UCH-L1 in the AD brain, which in turn impairs BDNF/TrkB-mediated retrograde signaling, compromising synaptic plasticity and neuronal survival. ..
  65. Mtango N, Sutovsky M, Vandevoort C, Latham K, Sutovsky P. Essential role of ubiquitin C-terminal hydrolases UCHL1 and UCHL3 in mammalian oocyte maturation. J Cell Physiol. 2012;227:2022-9 pubmed publisher
    ..This study examined the roles of two oocyte-expressed UCHs, UCHL1, and UCHL3 in murine and rhesus monkey oocyte maturation...
  66. CHALAZONITIS A, Tang A, Shang Y, Pham T, Hsieh I, Setlik W, et al. Homeodomain interacting protein kinase 2 regulates postnatal development of enteric dopaminergic neurons and glia via BMP signaling. J Neurosci. 2011;31:13746-57 pubmed publisher
  67. Peters E, Botchkarev V, Müller Röver S, Moll I, Rice F, Paus R. Developmental timing of hair follicle and dorsal skin innervation in mice. J Comp Neurol. 2002;448:28-52 pubmed
    ..This sequence of developing innervation consistently correlates with hair follicle development, indicating a close interdependence of neuronal and epithelial morphogenesis. ..
  68. Yamazaki K, Moriya H, Ichihara N, Mitsushio H, Inagaki S, Kikuchi T. Substance P-immunoreactive astrocytes in gracile sensory nervous tract of spinal cord in gracile axonal dystrophy mutant mouse. Mol Chem Neuropathol. 1993;20:1-20 pubmed
    In the gracile axonal dystrophy (GAD) mutant mouse, the dying-back type axonal dystrophy of the primary afferent neurons in the gracile tract of the spinal cord was marked by severe gliosis characterized by the hypertrophy and ..
  69. Tarozzo G, Cappello P, De Andrea M, Walters E, Margolis F, Oestreicher B, et al. Prenatal differentiation of mouse vomeronasal neurones. Eur J Neurosci. 1998;10:392-6 pubmed
  70. Christianson M, Gerolstein A, Lee H, Monseur B, Robinson D, Evans J. Effects of Ubiquitin C-Terminal Hydrolase L1 (UCH-L1) inhibition on sperm incorporation and cortical tension in mouse eggs. Mol Reprod Dev. 2016;83:188-9 pubmed publisher
  71. Løes S, Kettunen P, Kvinnsland H, Luukko K. Mouse rudimentary diastema tooth primordia are devoid of peripheral nerve fibers. Anat Embryol (Berl). 2002;205:187-91 pubmed
  72. Hiltunen J, Laurikainen A, Airaksinen M, Saarma M. GDNF family receptors in the embryonic and postnatal rat heart and reduced cholinergic innervation in mice hearts lacking ret or GFRalpha2. Dev Dyn. 2000;219:28-39 pubmed
    ..These findings indicate that GFRalpha2/Ret signaling is required for normal cholinergic innervation of heart. ..
  73. Abdo H, Li L, Lallemend F, Bachy I, Xu X, Rice F, et al. Dependence on the transcription factor Shox2 for specification of sensory neurons conveying discriminative touch. Eur J Neurosci. 2011;34:1529-41 pubmed publisher
    ..These results show that Shox2 is required for specification of a subclass of TrkB(+) sensory neurons which convey the sensation of discriminative touch arising from stimuli of the skin. ..
  74. Osbun N, Li J, O Driscoll M, Strominger Z, Wakahiro M, Rider E, et al. Genetic and functional analyses identify DISC1 as a novel callosal agenesis candidate gene. Am J Med Genet A. 2011;155A:1865-76 pubmed publisher
    ..Taken together our results suggest a significant role for DISC1 in corpus callosum development. ..
  75. Yao Y, Shi Y, Jia Z, Jiang Y, Gu Z, Wang J, et al. PTOV1 is associated with UCH-L1 and in response to estrogen stimuli during the mouse oocyte development. Histochem Cell Biol. 2011;136:205-15 pubmed publisher
    ..Moreover, the discovery that PTOV1 is associated with UCH-L1 in mouse oocytes supports the explanations for that UCH-L1 is involved in oocyte development and maturation, especially under the regulation of estrogen. ..
  76. Bolon B, Jing S, Asuncion F, Scully S, Pisegna M, Van G, et al. The candidate neuroprotective agent artemin induces autonomic neural dysplasia without preventing peripheral nerve dysfunction. Toxicol Pathol. 2004;32:275-94 pubmed
  77. Maka M, Stolt C, Wegner M. Identification of Sox8 as a modifier gene in a mouse model of Hirschsprung disease reveals underlying molecular defect. Dev Biol. 2005;277:155-69 pubmed
    ..Our study suggests that Sox8 and Sox10 are jointly required for the maintenance of these vagal neural crest stem cells. ..
  78. van Tuyl M, Groenman F, Kuliszewski M, Ridsdale R, Wang J, Tibboel D, et al. Overexpression of lunatic fringe does not affect epithelial cell differentiation in the developing mouse lung. Am J Physiol Lung Cell Mol Physiol. 2005;288:L672-82 pubmed
    ..Collectively, the data suggest that Lunatic fringe does not play a significant role in determining cell fate in fetal airway epithelium. ..
  79. Feng W, Simoes de Souza F, Finger T, Restrepo D, Williams T. Disorganized olfactory bulb lamination in mice deficient for transcription factor AP-2epsilon. Mol Cell Neurosci. 2009;42:161-71 pubmed publisher
    ..We also show that Cre recombinase expression directed by the AP-2epsilon locus can specifically target floxed genes within the olfactory bulb, extending the utility of this AK allele. ..
  80. Kameda Y. Mash1 is required for glomus cell formation in the mouse carotid body. Dev Biol. 2005;283:128-39 pubmed
    ..Many cells immunoreactive for the S-100 protein, a sustentacular cell marker, appear in the mutant carotid body during fetal development. The Mash1 gene is thus required for the genesis of glomus cells but not for sustentacular cells. ..
  81. Kwon J, Sekiguchi S, Wang Y, Setsuie R, Yoshikawa Y, Wada K. The region-specific functions of two ubiquitin C-terminal hydrolase isozymes along the epididymis. Exp Anim. 2006;55:35-43 pubmed
    We previously showed that gad mice, which are deficient for ubiquitin C-terminal hydrolase L1 (UCH-L1), have a significantly increased number of defective spermatozoa, suggesting that UCH-L1 functions in sperm quality control during ..
  82. Cheung K, Mok S, Rezaie P, Chan W. Dynamic expression of Dab2 in the mouse embryonic central nervous system. BMC Dev Biol. 2008;8:76 pubmed publisher
    ..This is the first study to demonstrate the dynamic spatiotemporal expression of Dab2 protein within the CNS during development. ..
  83. Koma Y, Furuno T, Hagiyama M, Hamaguchi K, Nakanishi M, Masuda M, et al. Cell adhesion molecule 1 is a novel pancreatic-islet cell adhesion molecule that mediates nerve-islet cell interactions. Gastroenterology. 2008;134:1544-54 pubmed publisher
    ..An immunohistochemical screen showed that CADM1 is expressed in pancreatic islets. Here, we determined the cell types expressing CADM1 and examined its role in nerve-islet cell interactions...