Type |
Details |
Score |
Gene |
Type: |
gene |
Organism: |
frog, western clawed |
|
•
•
•
•
•
|
Gene |
Type: |
gene |
Organism: |
human |
|
•
•
•
•
•
|
Gene |
Type: |
gene |
Organism: |
dog, domestic |
|
•
•
•
•
•
|
Gene |
Type: |
gene |
Organism: |
chicken |
|
•
•
•
•
•
|
Gene |
|
•
•
•
•
•
|
Gene |
Type: |
gene |
Organism: |
chimpanzee |
|
•
•
•
•
•
|
Gene |
Type: |
gene |
Organism: |
cattle |
|
•
•
•
•
•
|
Gene |
Type: |
gene |
Organism: |
zebrafish |
|
•
•
•
•
•
|
Gene |
Type: |
gene |
Organism: |
macaque, rhesus |
|
•
•
•
•
•
|
Protein Coding Gene |
Type: |
protein_coding_gene |
Organism: |
mouse, laboratory |
|
•
•
•
•
•
|
Protein Domain |
Type: |
Family |
Description: |
Vacuolar protein sorting-associated protein 54 (Vps54) is a component of the GARP (Vps52/53/54) complex that is involved in retrograde transport from early and late endosomes to the trans-Golgi network (TGN) []. Mammalian GARP complex has been shown to play a general role in the delivery of retrograde cargo into the TGN []. In mice, a point mutation (L967Q) in the Vps54 gene causes wobbler mutation, which is characterised by degeneration of upper and lower motor neurons leading to progressive muscle weakness. The wobbler mouse is an animal model for human motor neuron disease, such as amyotrophic lateral sclerosis (ALS) []. Interestingly, the protein mutation (L967Q) does not prevent its integration into the GARP complex but greatly reduces the half-life and levels of the protein in vivo []. |
|
•
•
•
•
•
|
Publication |
First Author: |
Pérez-Victoria FJ |
Year: |
2010 |
Journal: |
Proc Natl Acad Sci U S A |
Title: |
Structural basis for the wobbler mouse neurodegenerative disorder caused by mutation in the Vps54 subunit of the GARP complex. |
Volume: |
107 |
Issue: |
29 |
Pages: |
12860-5 |
|
•
•
•
•
•
|
Publication |
First Author: |
Schmitt-John T |
Year: |
2005 |
Journal: |
Nat Genet |
Title: |
Mutation of Vps54 causes motor neuron disease and defective spermiogenesis in the wobbler mouse. |
Volume: |
37 |
Issue: |
11 |
Pages: |
1213-5 |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
278
 |
Fragment?: |
true |
|
•
•
•
•
•
|
Protein |
Organism: |
Mus musculus/domesticus |
Length: |
685
 |
Fragment?: |
false |
|
•
•
•
•
•
|
Publication |
First Author: |
Gioria M |
Year: |
2017 |
Journal: |
Cell Tissue Res |
Title: |
Dynamic of contribution of UBPy-sorted cargo to acrosome biogenesis: effects of its derailment in a mouse model of globozoospermia, the infertile Vps54 (L967Q) mutant. |
Volume: |
369 |
Issue: |
2 |
Pages: |
413-427 |
|
•
•
•
•
•
|
Publication |
First Author: |
Karlsson P |
Year: |
2013 |
Journal: |
Int J Mol Sci |
Title: |
Loss of vps54 function leads to vesicle traffic impairment, protein mis-sorting and embryonic lethality. |
Volume: |
14 |
Issue: |
6 |
Pages: |
10908-25 |
|
•
•
•
•
•
|
Publication |
First Author: |
Walter L |
Year: |
2002 |
Journal: |
Gene |
Title: |
Identification, characterization and cytogenetic mapping of a yeast Vps54 homolog in rat and mouse. |
Volume: |
285 |
Issue: |
1-2 |
Pages: |
213-20 |
|
•
•
•
•
•
|
Publication |
First Author: |
Schmitt-John T |
Year: |
2015 |
Journal: |
Front Neurosci |
Title: |
VPS54 and the wobbler mouse. |
Volume: |
9 |
|
Pages: |
381 |
|
•
•
•
•
•
|
Publication |
First Author: |
Petit CS |
Year: |
2020 |
Journal: |
Proc Natl Acad Sci U S A |
Title: |
Inhibition of sphingolipid synthesis improves outcomes and survival in GARP mutant wobbler mice, a model of motor neuron degeneration. |
Volume: |
117 |
Issue: |
19 |
Pages: |
10565-10574 |
|
•
•
•
•
•
|
Publication |
First Author: |
Gonzalez Deniselle MC |
Year: |
2012 |
Journal: |
J Neurochem |
Title: |
Progesterone prevents mitochondrial dysfunction in the spinal cord of wobbler mice. |
Volume: |
122 |
Issue: |
1 |
Pages: |
185-95 |
|
•
•
•
•
•
|
Publication |
First Author: |
Duchen LW |
Year: |
1968 |
Journal: |
J Neurol Neurosurg Psychiatry |
Title: |
An hereditary motor neurone disease with progressive denervation of muscle in the mouse: the mutant 'wobbler'. |
Volume: |
31 |
Issue: |
6 |
Pages: |
535-42 |
|
•
•
•
•
•
|
Publication |
First Author: |
Santoro B |
Year: |
2004 |
Journal: |
Neurobiol Dis |
Title: |
Evidence for chronic mitochondrial impairment in the cervical spinal cord of a murine model of motor neuron disease. |
Volume: |
17 |
Issue: |
2 |
Pages: |
349-57 |
|
•
•
•
•
•
|
Publication |
First Author: |
Clowry GJ |
Year: |
2004 |
Journal: |
Neuropathol Appl Neurobiol |
Title: |
Brainstem motor nuclei respond differentially to degenerative disease in the mutant mouse wobbler. |
Volume: |
30 |
Issue: |
2 |
Pages: |
148-60 |
|
•
•
•
•
•
|
Publication |
First Author: |
Meyer M |
Year: |
2015 |
Journal: |
Neuroscience |
Title: |
The progesterone receptor agonist Nestorone holds back proinflammatory mediators and neuropathology in the wobbler mouse model of motoneuron degeneration. |
Volume: |
308 |
|
Pages: |
51-63 |
|
•
•
•
•
•
|
Publication |
First Author: |
Ikeda K |
Year: |
1998 |
Journal: |
J Neurol Sci |
Title: |
Neuronal nitric oxide synthase inhibitor, 7-nitroindazole, delays motor dysfunction and spinal motoneuron degeneration in the wobbler mouse. |
Volume: |
160 |
Issue: |
1 |
Pages: |
9-15 |
|
•
•
•
•
•
|
Publication |
First Author: |
Blondet B |
Year: |
1992 |
Journal: |
Brain Res |
Title: |
Plasminogen activators in the neuromuscular system of the wobbler mutant mouse. |
Volume: |
580 |
Issue: |
1-2 |
Pages: |
303-10 |
|
•
•
•
•
•
|
Publication |
First Author: |
Junghans M |
Year: |
2022 |
Journal: |
Front Cell Neurosci |
Title: |
ROS scavengers decrease ÎłH2ax spots in motor neuronal nuclei of ALS model mice in vitro. |
Volume: |
16 |
|
Pages: |
963169 |
|
•
•
•
•
•
|
Publication |
First Author: |
Dennis JS |
Year: |
2009 |
Journal: |
Neuroscience |
Title: |
Wobbler mice modeling motor neuron disease display elevated transactive response DNA binding protein. |
Volume: |
158 |
Issue: |
2 |
Pages: |
745-50 |
|
•
•
•
•
•
|
Publication |
First Author: |
Bigini P |
Year: |
2001 |
Journal: |
Neuroreport |
Title: |
Glutamate transporters in the spinal cord of the wobbler mouse. |
Volume: |
12 |
Issue: |
9 |
Pages: |
1815-20 |
|
•
•
•
•
•
|
Publication |
First Author: |
Corvino V |
Year: |
2003 |
Journal: |
Neurochem Res |
Title: |
S100B protein and 4-hydroxynonenal in the spinal cord of wobbler mice. |
Volume: |
28 |
Issue: |
2 |
Pages: |
341-5 |
|
•
•
•
•
•
|
Publication |
First Author: |
Tomiyama M |
Year: |
1994 |
Journal: |
Brain Res |
Title: |
Quantitative autoradiographic distribution of glutamate receptors in the cervical segment of the spinal cord of the wobbler mouse. |
Volume: |
650 |
Issue: |
2 |
Pages: |
353-7 |
|
•
•
•
•
•
|
Publication |
First Author: |
Murakami T |
Year: |
1980 |
Journal: |
Exp Neurol |
Title: |
Reduced protein synthesis in spinal anterior horn neurons in wobbler mouse mutant. |
Volume: |
67 |
Issue: |
2 |
Pages: |
423-32 |
|
•
•
•
•
•
|
Publication |
First Author: |
Thielsen KD |
Year: |
2013 |
Journal: |
PLoS One |
Title: |
The Wobbler mouse model of amyotrophic lateral sclerosis (ALS) displays hippocampal hyperexcitability, and reduced number of interneurons, but no presynaptic vesicle release impairments. |
Volume: |
8 |
Issue: |
12 |
Pages: |
e82767 |
|
•
•
•
•
•
|
Publication |
First Author: |
Blondet B |
Year: |
2001 |
Journal: |
Neurosci Lett |
Title: |
Transient massive DNA fragmentation in nervous system during the early course of a murine neurodegenerative disease. |
Volume: |
305 |
Issue: |
3 |
Pages: |
202-6 |
|
•
•
•
•
•
|
Publication |
First Author: |
Bose P |
Year: |
1998 |
Journal: |
Brain Res |
Title: |
A novel behavioral method to detect motoneuron disease in Wobbler mice aged three to seven days old. |
Volume: |
813 |
Issue: |
2 |
Pages: |
334-42 |
|
•
•
•
•
•
|
Publication |
First Author: |
Mitsumoto H |
Year: |
1994 |
Journal: |
Science |
Title: |
Arrest of motor neuron disease in wobbler mice cotreated with CNTF and BDNF. |
Volume: |
265 |
Issue: |
5175 |
Pages: |
1107-10 |
|
•
•
•
•
•
|
Publication |
First Author: |
Blondet B |
Year: |
2002 |
Journal: |
Brain Res |
Title: |
Motoneuron morphological alterations before and after the onset of the disease in the wobbler mouse. |
Volume: |
930 |
Issue: |
1-2 |
Pages: |
53-7 |
|
•
•
•
•
•
|
Publication |
First Author: |
Bose P |
Year: |
1999 |
Journal: |
Brain Res Bull |
Title: |
Effects of assisted feeding on Wobbler mouse motoneuron disease and on serotonergic and peptidergic sprouting in the cervical spinal ventral horn. |
Volume: |
48 |
Issue: |
4 |
Pages: |
429-39 |
|
•
•
•
•
•
|
Publication |
First Author: |
Krieger C |
Year: |
1992 |
Journal: |
Can J Neurol Sci |
Title: |
Excitatory amino acid receptor antagonist in murine motoneuron disease (the wobbler mouse). |
Volume: |
19 |
Issue: |
4 |
Pages: |
462-5 |
|
•
•
•
•
•
|
Publication |
First Author: |
Ikeda K |
Year: |
2000 |
Journal: |
Brain Res |
Title: |
T-588, a novel neuroprotective agent, delays progression of neuromuscular dysfunction in wobbler mouse motoneuron disease. |
Volume: |
858 |
Issue: |
1 |
Pages: |
84-91 |
|
•
•
•
•
•
|
Publication |
First Author: |
Fumagalli E |
Year: |
2006 |
Journal: |
Exp Neurol |
Title: |
Riluzole, unlike the AMPA antagonist RPR119990, reduces motor impairment and partially prevents motoneuron death in the wobbler mouse, a model of neurodegenerative disease. |
Volume: |
198 |
Issue: |
1 |
Pages: |
114-28 |
|
•
•
•
•
•
|
Publication |
First Author: |
Dave KR |
Year: |
2003 |
Journal: |
Exp Neurol |
Title: |
Early mitochondrial dysfunction occurs in motor cortex and spinal cord at the onset of disease in the Wobbler mouse. |
Volume: |
182 |
Issue: |
2 |
Pages: |
412-20 |
|
•
•
•
•
•
|
Publication |
First Author: |
Mitsumoto H |
Year: |
1993 |
Journal: |
Muscle Nerve |
Title: |
Retardation of fast axonal transport in wobbler mice. |
Volume: |
16 |
Issue: |
5 |
Pages: |
542-7 |
|
•
•
•
•
•
|
Publication |
First Author: |
Meyer M |
Year: |
2020 |
Journal: |
Brain Res |
Title: |
Long-term effects of the glucocorticoid receptor modulator CORT113176 in murine motoneuron degeneration. |
Volume: |
1727 |
|
Pages: |
146551 |
|
•
•
•
•
•
|
Publication |
First Author: |
Hantaz-Ambroise D |
Year: |
1994 |
Journal: |
J Neurocytol |
Title: |
Abnormal astrocyte differentiation and defective cellular interactions in wobbler mouse spinal cord. |
Volume: |
23 |
Issue: |
3 |
Pages: |
179-92 |
|
•
•
•
•
•
|
Publication |
First Author: |
Brooks BR |
Year: |
1978 |
Journal: |
Arch Neurol |
Title: |
Decreased spinal cord cGMP in murine (wobbler) spontaneous lower motor neuron degeneration. |
Volume: |
35 |
Issue: |
9 |
Pages: |
590-1 |
|
•
•
•
•
•
|
Publication |
First Author: |
Hantaz-Ambroise D |
Year: |
2001 |
Journal: |
Differentiation |
Title: |
Specific features of chronic astrocyte gliosis after experimental central nervous system (CNS) xenografting and in Wobbler neurological mutant CNS. |
Volume: |
69 |
Issue: |
2-3 |
Pages: |
100-7 |
|
•
•
•
•
•
|
Publication |
First Author: |
Broch-Lips M |
Year: |
2013 |
Journal: |
Exp Neurol |
Title: |
Neuro-muscular function in the wobbler murine model of primary motor neuronopathy. |
Volume: |
248 |
|
Pages: |
406-15 |
|
•
•
•
•
•
|
Publication |
First Author: |
Ma WY |
Year: |
1991 |
Journal: |
J Comp Neurol |
Title: |
Reduced branching and length of dendrites detected in cervical spinal cord motoneurons of Wobbler mouse, a model for inherited motoneuron disease. |
Volume: |
311 |
Issue: |
2 |
Pages: |
210-22 |
|
•
•
•
•
•
|
Publication |
First Author: |
Bose P |
Year: |
1999 |
Journal: |
Neurosci Lett |
Title: |
Increase in fiber density for immunoreactive serotonin, substance P, enkephalin and thyrotropin-releasing hormone occurs during the early presymptomatic period of motoneuron disease in Wobbler mouse spinal cord ventral horn. |
Volume: |
260 |
Issue: |
3 |
Pages: |
196-200 |
|
•
•
•
•
•
|
Publication |
First Author: |
Vacca-Galloway LL |
Year: |
1986 |
Journal: |
J Neurosci Res |
Title: |
Substance P neurons sprout in the cervical spinal cord of the wobbler mouse: a model for motoneuron disease. |
Volume: |
16 |
Issue: |
4 |
Pages: |
657-70 |
|
•
•
•
•
•
|
Publication |
First Author: |
Andrews JM |
Year: |
1975 |
Journal: |
J Neuropathol Exp Neurol |
Title: |
The fine structure of the cervical spinal cord, ventral root and brachial nerves in the wobbler (wr) mouse. |
Volume: |
34 |
Issue: |
1 |
Pages: |
12-27 |
|
•
•
•
•
•
|
Publication |
First Author: |
Meccariello R |
Year: |
2002 |
Journal: |
Biol Reprod |
Title: |
Mouse sperm cell-specific DnaJ first homologue: an evolutionarily conserved protein for spermiogenesis. |
Volume: |
66 |
Issue: |
5 |
Pages: |
1328-35 |
|
•
•
•
•
•
|
Publication |
First Author: |
Augustin M |
Year: |
1997 |
Journal: |
Dev Dyn |
Title: |
Spinal muscular atrophy gene wobbler of the mouse: evidence from chimeric spinal cord and testis for cell-autonomous function. |
Volume: |
209 |
Issue: |
3 |
Pages: |
286-95 |
|
•
•
•
•
•
|
Publication |
First Author: |
Boillée S |
Year: |
2003 |
Journal: |
Mol Neurobiol |
Title: |
The wobbler mouse: a neurodegeneration jigsaw puzzle. |
Volume: |
28 |
Issue: |
1 |
Pages: |
65-106 |
|
•
•
•
•
•
|
Publication |
First Author: |
González Deniselle MC |
Year: |
2004 |
Journal: |
Brain Res |
Title: |
Progesterone treatment reduces NADPH-diaphorase/nitric oxide synthase in Wobbler mouse motoneuron disease. |
Volume: |
1014 |
Issue: |
1-2 |
Pages: |
71-9 |
|
•
•
•
•
•
|
Publication |
First Author: |
Leestma JE |
Year: |
1980 |
Journal: |
Am J Pathol |
Title: |
Animal model of human disease: Werdnig-Hoffmann disease (infantile spinal muscular atrophy). |
Volume: |
100 |
Issue: |
3 |
Pages: |
821-4 |
|
•
•
•
•
•
|
Publication |
First Author: |
Nieto-Gonzalez JL |
Year: |
2011 |
Journal: |
Cereb Cortex |
Title: |
Reduced GABAergic inhibition explains cortical hyperexcitability in the wobbler mouse model of ALS. |
Volume: |
21 |
Issue: |
3 |
Pages: |
625-35 |
|
•
•
•
•
•
|
Publication |
First Author: |
Ma W |
Year: |
1992 |
Journal: |
Restor Neurol Neurosci |
Title: |
Spiny interneurons identified in the normal mouse spinal cord show alterations in the Wobbler mouse: a model for inherited motoneuron disease. |
Volume: |
4 |
Issue: |
6 |
Pages: |
381-92 |
|
•
•
•
•
•
|
Publication |
First Author: |
Shi J |
Year: |
1993 |
Journal: |
Brain Res |
Title: |
Thyrotropin-releasing hormone (TRH) neurons sprout in cervical spinal cord of Wobbler mouse. |
Volume: |
626 |
Issue: |
1-2 |
Pages: |
83-9 |
|
•
•
•
•
•
|
Publication |
First Author: |
Toursel T |
Year: |
2000 |
Journal: |
Exp Neurol |
Title: |
Alterations in contractile properties and expression of myofibrillar proteins in wobbler mouse muscles. |
Volume: |
162 |
Issue: |
2 |
Pages: |
311-20 |
|
•
•
•
•
•
|
Publication |
First Author: |
Zhang YQ |
Year: |
1992 |
Journal: |
Brain Res |
Title: |
Decreased immunoreactive (IR) calcitonin gene-related peptide correlates with sprouting of IR-peptidergic and serotonergic neuronal processes in spinal cord and brain nuclei from the Wobbler mouse during motoneuron disease. |
Volume: |
587 |
Issue: |
1 |
Pages: |
169-77 |
|
•
•
•
•
•
|
Publication |
First Author: |
Ikeda K |
Year: |
1996 |
Journal: |
Muscle Nerve |
Title: |
Basic fibroblast growth factor has neuroprotective effects on axotomy-induced spinal motoneuron death and wobbler mouse motoneuron disease. |
Volume: |
19 |
Issue: |
6 |
Pages: |
794-5 |
|
•
•
•
•
•
|
Publication |
First Author: |
Ikeda K |
Year: |
1995 |
Journal: |
Neurol Res |
Title: |
Neuroprotective effect of basic fibroblast growth factor on wobbler mouse motor neuron disease. |
Volume: |
17 |
Issue: |
6 |
Pages: |
445-8 |
|
•
•
•
•
•
|
Publication |
First Author: |
Yung KK |
Year: |
1994 |
Journal: |
Brain Res |
Title: |
Alterations in acetylcholinesterase and choline acetyltransferase activities and neuropeptide levels in the ventral spinal cord of the Wobbler mouse during inherited motoneuron disease. |
Volume: |
638 |
Issue: |
1-2 |
Pages: |
337-42 |
|
•
•
•
•
•
|
Publication |
First Author: |
Ishiyama T |
Year: |
1997 |
Journal: |
Exp Neurol |
Title: |
Genetic transfer of the wobbler gene to a C57BL/6J x NZB hybrid stock: natural history of the motor neuron disease and response to CNTF and BDNF cotreatment. |
Volume: |
148 |
Issue: |
1 |
Pages: |
247-55 |
|
•
•
•
•
•
|
Publication |
First Author: |
Saberi D |
Year: |
2016 |
Journal: |
J Neuropathol Exp Neurol |
Title: |
The Spatiotemporal Pattern of Degeneration in the Cerebellum of the Wobbler Mouse. |
Volume: |
75 |
Issue: |
4 |
Pages: |
347-57 |
|
•
•
•
•
•
|
Publication |
First Author: |
Pollin MM |
Year: |
1990 |
Journal: |
J Neurocytol |
Title: |
Loss of motor neurons from the median nerve motor nucleus of the mutant mouse 'wobbler'. |
Volume: |
19 |
Issue: |
1 |
Pages: |
29-38 |
|
•
•
•
•
•
|
Publication |
First Author: |
González Deniselle MC |
Year: |
1997 |
Journal: |
J Steroid Biochem Mol Biol |
Title: |
Glucocorticoid receptors and actions in the spinal cord of the Wobbler mouse, a model for neurodegenerative diseases. |
Volume: |
60 |
Issue: |
3-4 |
Pages: |
205-13 |
|
•
•
•
•
•
|
Publication |
First Author: |
Dockery P |
Year: |
1997 |
Journal: |
J Anat |
Title: |
Neuron volume in the ventral horn in Wobbler mouse motoneuron disease: a light microscope stereological study. |
Volume: |
191 ( Pt 1) |
|
Pages: |
89-98 |
|
•
•
•
•
•
|
Publication |
First Author: |
Yung KK |
Year: |
1992 |
Journal: |
Brain Res |
Title: |
Decrease of enkephalins in cerebellum during Wobbler mouse motoneuron disease. |
Volume: |
599 |
Issue: |
1 |
Pages: |
175-80 |
|
•
•
•
•
•
|
Publication |
First Author: |
Mitsumoto H |
Year: |
1986 |
Journal: |
Ann Neurol |
Title: |
Impaired slow axonal transport in wobbler mouse motor neuron disease. |
Volume: |
19 |
Issue: |
1 |
Pages: |
36-43 |
|
•
•
•
•
•
|
Publication |
First Author: |
Heimann P |
Year: |
1991 |
Journal: |
Differentiation |
Title: |
Defect of sperm assembly in a neurological mutant of the mouse, wobbler (WR). |
Volume: |
47 |
Issue: |
2 |
Pages: |
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