Literature DB >> 26583493

Expanding Phenotype of VRK1 Mutations in Motor Neuron Disease.

Thy P Nguyen1, Suur Biliciler, Wojciech Wiszniewski, Kazim Sheikh.   

Abstract

OBJECTIVE: In the past decade, hereditary forms of motor neuron disease (spinal muscular atrophy and/or amyotrophic lateral sclerosis) are increasingly identified. As advanced genetic testing is performed, molecular diagnosis can be obtained. Identifying new gene mutations can lead to further understanding of disease. METHODS AND
RESULTS: We report a single case of a patient with early-onset amyotrophic lateral sclerosis, evaluated at University of Texas Health Houston Science Center from 2011-2014. Initial genetic testing did not reveal an etiology in this patient. Through whole-exome sequencing, a VRK1 mutation was identified. CONCLUSIONS AND RELEVANCE: We identify a possible new cause of hereditary amyotrophic lateral sclerosis, VRK1 mutation. This case report also expands the phenotypic spectrum of this mutation in neurologic diseases.

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Year:  2015        PMID: 26583493      PMCID: PMC4829393          DOI: 10.1097/CND.0000000000000096

Source DB:  PubMed          Journal:  J Clin Neuromuscul Dis        ISSN: 1522-0443


  8 in total

Review 1.  El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis.

Authors:  B R Brooks; R G Miller; M Swash; T L Munsat
Journal:  Amyotroph Lateral Scler Other Motor Neuron Disord       Date:  2000-12

2.  Deep sequencing reveals 50 novel genes for recessive cognitive disorders.

Authors:  Hossein Najmabadi; Hao Hu; Masoud Garshasbi; Tomasz Zemojtel; Seyedeh Sedigheh Abedini; Wei Chen; Masoumeh Hosseini; Farkhondeh Behjati; Stefan Haas; Payman Jamali; Agnes Zecha; Marzieh Mohseni; Lucia Püttmann; Leyla Nouri Vahid; Corinna Jensen; Lia Abbasi Moheb; Melanie Bienek; Farzaneh Larti; Ines Mueller; Robert Weissmann; Hossein Darvish; Klaus Wrogemann; Valeh Hadavi; Bettina Lipkowitz; Sahar Esmaeeli-Nieh; Dagmar Wieczorek; Roxana Kariminejad; Saghar Ghasemi Firouzabadi; Monika Cohen; Zohreh Fattahi; Imma Rost; Faezeh Mojahedi; Christoph Hertzberg; Atefeh Dehghan; Anna Rajab; Mohammad Javad Soltani Banavandi; Julia Hoffer; Masoumeh Falah; Luciana Musante; Vera Kalscheuer; Reinhard Ullmann; Andreas Walter Kuss; Andreas Tzschach; Kimia Kahrizi; H Hilger Ropers
Journal:  Nature       Date:  2011-09-21       Impact factor: 49.962

Review 3.  The genetics of spinal muscular atrophies.

Authors:  Claribel D Wee; Lingling Kong; Charlotte J Sumner
Journal:  Curr Opin Neurol       Date:  2010-10       Impact factor: 5.710

4.  Mutations in VRK1 associated with complex motor and sensory axonal neuropathy plus microcephaly.

Authors:  Claudia Gonzaga-Jauregui; Timothy Lotze; Leila Jamal; Samantha Penney; Ian M Campbell; Davut Pehlivan; Jill V Hunter; Suzanne L Woodbury; Gerald Raymond; Adekunle M Adesina; Shalini N Jhangiani; Jeffrey G Reid; Donna M Muzny; Eric Boerwinkle; James R Lupski; Richard A Gibbs; Wojciech Wiszniewski
Journal:  JAMA Neurol       Date:  2013-12       Impact factor: 18.302

5.  p53 downregulates its activating vaccinia-related kinase 1, forming a new autoregulatory loop.

Authors:  Alberto Valbuena; Francisco M Vega; Sandra Blanco; Pedro A Lazo
Journal:  Mol Cell Biol       Date:  2006-07       Impact factor: 4.272

6.  p53 is abnormally elevated and active in the CNS of patients with amyotrophic lateral sclerosis.

Authors:  L J Martin
Journal:  Neurobiol Dis       Date:  2000-12       Impact factor: 5.996

7.  A direct interaction between the survival motor neuron protein and p53 and its relationship to spinal muscular atrophy.

Authors:  Philip J Young; Patricia M Day; Jianhua Zhou; Elliot J Androphy; Glenn E Morris; Christian L Lorson
Journal:  J Biol Chem       Date:  2001-11-09       Impact factor: 5.157

8.  Spinal muscular atrophy with pontocerebellar hypoplasia is caused by a mutation in the VRK1 gene.

Authors:  Paul Renbaum; Efrat Kellerman; Ranit Jaron; Dan Geiger; Reeval Segel; Ming Lee; Mary Claire King; Ephrat Levy-Lahad
Journal:  Am J Hum Genet       Date:  2009-07-30       Impact factor: 11.025

  8 in total
  13 in total

1.  Dysregulation of Cellular VRK1, BAF, and Innate Immune Signaling by the Vaccinia Virus B12 Pseudokinase.

Authors:  Alexandria C Linville; Amber B Rico; Helena Teague; Lucy E Binsted; Geoffrey L Smith; Jonas D Albarnaz; Matthew S Wiebe
Journal:  J Virol       Date:  2022-05-11       Impact factor: 6.549

2.  Multivalent DNA and nucleosome acidic patch interactions specify VRK1 mitotic localization and activity.

Authors:  Gabrielle R Budziszewski; Yani Zhao; Cathy J Spangler; Katarzyna M Kedziora; Michael R Williams; Dalal N Azzam; Aleksandra Skrajna; Yuka Koyama; Andrew P Cesmat; Holly C Simmons; Eyla C Arteaga; Joshua D Strauss; Dmitri Kireev; Robert K McGinty
Journal:  Nucleic Acids Res       Date:  2022-05-06       Impact factor: 19.160

3.  VRK1 (Y213H) homozygous mutant impairs Cajal bodies in a hereditary case of distal motor neuropathy.

Authors:  Ana T Marcos; Elena Martín-Doncel; Patricia Morejón-García; Iñigo Marcos-Alcalde; Paulino Gómez-Puertas; María Segura-Puimedon; Lluis Armengol; José M Navarro-Pando; Pedro A Lazo
Journal:  Ann Clin Transl Neurol       Date:  2020-05-04       Impact factor: 4.511

4.  Vrk1 partial Knockdown in Mice Results in Reduced Brain Weight and Mild Motor Dysfunction, and Indicates Neuronal VRK1 Target Pathways.

Authors:  Hadar Vinograd-Byk; Paul Renbaum; Ephrat Levy-Lahad
Journal:  Sci Rep       Date:  2018-07-26       Impact factor: 4.379

5.  A novel VRK1 mutation associated with recessive distal hereditary motor neuropathy.

Authors:  Shu-Yan Feng; Liu-Yi Li; Shu-Man Feng; Zhang-Yu Zou
Journal:  Ann Clin Transl Neurol       Date:  2018-12-03       Impact factor: 4.511

6.  Motor neuron diseases caused by a novel VRK1 variant - A genotype/phenotype study.

Authors:  Maryam Sedghi; Ali-Reza Moslemi; Montse Olive; Masoud Etemadifar; Behnaz Ansari; Jafar Nasiri; Leila Emrahi; Hamid-Reza Mianesaz; Nigel G Laing; Homa Tajsharghi
Journal:  Ann Clin Transl Neurol       Date:  2019-09-27       Impact factor: 4.511

Review 7.  Recent advances in the genetic neuropathies.

Authors:  Alexander M Rossor; Pedro J Tomaselli; Mary M Reilly
Journal:  Curr Opin Neurol       Date:  2016-10       Impact factor: 5.710

8.  Adult-Onset Spinal Muscular Atrophy due to Mutations in the VRK1 Gene.

Authors:  Angela Sung; Paolo Moretti; Aziz Shaibani
Journal:  Neurol Genet       Date:  2021-06-22

9.  Novel motor phenotypes in patients with VRK1 mutations without pontocerebellar hypoplasia.

Authors:  Marion Stoll; Hooiling Teoh; James Lee; Stephen Reddel; Ying Zhu; Michael Buckley; Hugo Sampaio; Tony Roscioli; Michelle Farrar; Garth Nicholson
Journal:  Neurology       Date:  2016-06-08       Impact factor: 9.910

10.  VRK1 functional insufficiency due to alterations in protein stability or kinase activity of human VRK1 pathogenic variants implicated in neuromotor syndromes.

Authors:  Elena Martín-Doncel; Ana M Rojas; Lara Cantarero; Pedro A Lazo
Journal:  Sci Rep       Date:  2019-09-16       Impact factor: 4.379

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