Literature DB >> 3340629

Gracile axonal dystrophy (GAD), a new neurological mutant in the mouse.

K Yamazaki1, N Wakasugi, T Tomita, T Kikuchi, M Mukoyama, K Ando.   

Abstract

A new neurological mutant has been found in the F2 offspring of CBA/Nga and RFM/Nga mice. Affected mice exhibited ataxia beginning at about 80 days of age, followed by tremor, difficulty in moving, and muscular atrophy of the hind limbs. The neurological signs became progressively severe, and death occurred by 5 to 6 months of age. Since the animals could be distinguished from normal mice by the abnormal positions of the hind limbs when the mouse was hung by the tail after 1 month of age, they could be bred until onset of the signs. Pathological examination revealed neuroaxonal dystrophy and degeneration in the gracile nucleus of the medulla oblongata and the gracile fascicules of the spinal cord, which could be the main cause of the clinical signs. The mutation is inherited as an autosomal recessive trait. It was, therefore, named gracile axonal dystrophy (GAD) with the gene symbol gad. The mice could be a new pathological model for the study of neuroaxonal dystrophy.

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Year:  1988        PMID: 3340629     DOI: 10.3181/00379727-187-42656

Source DB:  PubMed          Journal:  Proc Soc Exp Biol Med        ISSN: 0037-9727


  34 in total

1.  Porcine UCHL1: genomic organization, chromosome localization and expression analysis.

Authors:  Knud Larsen; Lone Bruhn Madsen; Christian Bendixen
Journal:  Mol Biol Rep       Date:  2011-05-13       Impact factor: 2.316

2.  Fine structural changes of muscle spindles in the gracile axonal dystrophy mutant mouse.

Authors:  A Takagi; K Oda; T Kikuchi; H Kajihara
Journal:  Virchows Arch       Date:  1996-07       Impact factor: 4.064

3.  Neuropathology of gracile axonal dystrophy (GAD) mouse. An animal model of central distal axonopathy in primary sensory neurons.

Authors:  M Mukoyama; K Yamazaki; T Kikuchi; T Tomita
Journal:  Acta Neuropathol       Date:  1989       Impact factor: 17.088

4.  Ubiquitin C-Terminal Hydrolase L1 regulates myoblast proliferation and differentiation.

Authors:  Hongbo Gao; Sigurd Hartnett; Yifan Li
Journal:  Biochem Biophys Res Commun       Date:  2017-08-10       Impact factor: 3.575

5.  Essential role of maternal UCHL1 and UCHL3 in fertilization and preimplantation embryo development.

Authors:  Namdori R Mtango; Miriam Sutovsky; Andrej Susor; Zhisheng Zhong; Keith E Latham; Peter Sutovsky
Journal:  J Cell Physiol       Date:  2012-04       Impact factor: 6.384

6.  Ubiquitin C-terminal hydrolase L1 is required for pancreatic beta cell survival and function in lipotoxic conditions.

Authors:  K Y Chu; H Li; K Wada; J D Johnson
Journal:  Diabetologia       Date:  2011-10-25       Impact factor: 10.122

7.  Parkin-mediated K63-polyubiquitination targets ubiquitin C-terminal hydrolase L1 for degradation by the autophagy-lysosome system.

Authors:  Jeanne E McKeon; Di Sha; Lian Li; Lih-Shen Chin
Journal:  Cell Mol Life Sci       Date:  2014-11-18       Impact factor: 9.261

8.  Ubiquitin C-terminal hydrolase L1 interacts with choline transporter in cholinergic cells.

Authors:  Sigurd Hartnett; Fan Zhang; Allison Abitz; Yifan Li
Journal:  Neurosci Lett       Date:  2014-02-11       Impact factor: 3.046

9.  Ubiquitin carboxyl-terminal hydrolase L1 is required for maintaining the structure and function of the neuromuscular junction.

Authors:  Fujun Chen; Yoshie Sugiura; Kalisa Galina Myers; Yun Liu; Weichun Lin
Journal:  Proc Natl Acad Sci U S A       Date:  2010-01-04       Impact factor: 11.205

10.  Recessive loss of function of the neuronal ubiquitin hydrolase UCHL1 leads to early-onset progressive neurodegeneration.

Authors:  Kaya Bilguvar; Navneet K Tyagi; Cigdem Ozkara; Beyhan Tuysuz; Mehmet Bakircioglu; Murim Choi; Sakir Delil; Ahmet O Caglayan; Jacob F Baranoski; Ozdem Erturk; Cengiz Yalcinkaya; Murat Karacorlu; Alp Dincer; Michele H Johnson; Shrikant Mane; Sreeganga S Chandra; Angeliki Louvi; Titus J Boggon; Richard P Lifton; Arthur L Horwich; Murat Gunel
Journal:  Proc Natl Acad Sci U S A       Date:  2013-01-28       Impact factor: 11.205

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