MCID: NRN016
MIFTS: 39

Neuronal Migration Disorders malady

Neuronal category

Summaries for Neuronal Migration Disorders

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44NINDS, 64Wikipedia, 33MalaCards
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NINDS:44 Neuronal migration disorders (NMDs) are a group of birth defects caused by the abnormal migration of neurons in the developing brain and nervous system. In the developing brain, neurons must migrate from the areas where they are born to the areas where they will settle into their proper neural circuits. Neuronal migration, which occurs as early as the second month of gestation, is controlled by a complex assortment of chemical guides and signals. When these signals are absent or incorrect, neurons do not end up where they belong. This can result in structurally abnormal or missing areas of the brain in the cerebral hemispheres, cerebellum, brainstem, or hippocampus. The structural abnormalities found in NMDs include schizencephaly, porencephaly, lissencephaly, agyria, macrogyria, polymicrogyria, pachygyria, microgyria, micropolygyria, neuronal heterotopias (including band heterotopia), agenesis of the corpus callosum, and agenesis of the cranial nerves. Symptoms vary according to the abnormality, but often feature poor muscle tone and motor function, seizures, developmental delays, mental retardation, failure to grow and thrive, difficulties with feeding, swelling in the extremities, and a smaller than normal head. Most infants with an NMD appear normal, but some disorders have characteristic facial or skull features that can be recognized by a neurologist. Several genetic abnormalities in children with NMDs have been identified. Defects in genes that are involved in neuronal migration have been associated with NMDs, but the role they play in the development of these disorders is not yet well-understood. More than 25 syndromes resulting from abnormal neuronal migration have been described. Among them are syndromes with several different patterns of inheritance; genetic counseling thus differs greatly between syndromes.

MalaCards: Neuronal Migration Disorders is related to zellweger syndrome and periventricular nodular heterotopia. An important gene associated with Neuronal Migration Disorders is ARX (aristaless related homeobox), and among its related pathways are Lissencephaly gene (LIS1) in neuronal migration and development and Neuroscience. The compounds nmda and cyanine have been mentioned in the context of this disorder. Affiliated tissues include skin, tongue and brain, and related mouse phenotypes are muscle and reproductive system.

Wikipedia:64 Nervous system disease refers to a general class of medical conditions affecting the nervous system. more...

Aliases & Classifications for Neuronal Migration Disorders

Sources:
44NINDS, 61UMLS
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Neuronal


Aliases & Descriptions:

neuronal migration disorders 44 61


Related Diseases for Neuronal Migration Disorders

Sources:
17GeneCards, 18GeneDecks
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Diseases related to Neuronal Migration Disorders via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 62)
idRelated DiseaseScoreTop Affiliating Genes
1zellweger syndrome30.3PEX2, DCX, NES
2periventricular nodular heterotopia30.3PAFAH1B1, GPR56, RELN, EMX2, DCX, FLNA
3pseudobulbar palsy30.1ARFGEF2
4walker-warburg syndrome30.1FKTN, POMGNT1, POMT1
5polymicrogyria30.1GPR56
6lissencephaly 130.1DCX, RELN, PAFAH1B1
7temporal lobe epilepsy30.1RELN, CALB1, PVALB
8mental retardation30.1LOC619409, ARX, FLNA, DCX, POMT1, POMGNT1
9lissencephaly30.1PAFAH1B1, GPR56, YWHAE, RELN, EMX2, FKTN
10n syndrome10.5
11seizure disorder10.4
12pachygyria10.2
13periventricular heterotopia10.2
14dysembryoplastic neuroepithelial tumor10.1
15dyslexia10.1
16diabetes insipidus10.1
17holoprosencephaly10.1
18turner syndrome10.1
19dwarfism10.1
20x-linked periventricular heterotopia10.1
21corpus callosum agenesis10.1
22agyria pachygyria polymicrogyria10.1
23colpocephaly10.1
24subcortical band heterotopia10.1
25knobloch syndrome10.1
26mental retardation epilepsy10.1
27subcortical laminar heterotopia10.1
28central bilateral macrogyria10.1
29pachygyria - intellectual deficit - epilepsy10.1
30alobar holoprosencephaly10.1
31retinitis10.0DCX
32dementia10.0MAP2, PVALB
33lissencephaly x-linked10.0ARX, DCX, PAFAH1B1
34muscular dystrophy-dystroglycanopathy , type a, 1410.0FKTN, POMGNT1, POMT1
35fukuyama type muscular dystrophy10.0POMT1, POMGNT1, FKTN
36hydrocephalus10.0POMGNT1, NES, FLNA
37limb-girdle muscular dystrophy10.0POMT1, POMGNT1, FKTN
38infantile epileptic encephalopathy10.0PAFAH1B1, FLNA, ARX
39tuberous sclerosis10.0NES, DCX, MAP2
40neurilemmoma10.0CALB2, NES, DCX
41brain ischemia10.0MAP2, NES
42creutzfeldt-jakob syndrome10.0NES, PVALB
43focal epilepsy10.0PAFAH1B1, GPR56, EMX2, DCX
44muscular dystrophy10.0FKTN, POMGNT1, POMT1, LOC619409
45microcephaly10.0PAFAH1B1, POMT1, ARFGEF2, LOC619409
46autistic disorder10.0ARX, RELN
47multiple system atrophy10.0PVALB, MAP2
48medulloblastoma10.0PAFAH1B1, MAP2, NES
49prion disease10.0MAP2, CALB2, PVALB
50cerebellar hypoplasia10.0DCX, RELN, PAFAH1B1

Graphical network of the top 20 diseases related to Neuronal Migration Disorders:



Diseases related to neuronal migration disorders

Clinical Features for Neuronal Migration Disorders

Drugs & Therapeutics for Neuronal Migration Disorders

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Neuronal Migration Disorders

Anatomical Context for Neuronal Migration Disorders

Sources:
33MalaCards
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MalaCards organs/tissues related to Neuronal Migration Disorders:

33
Skin, Tongue, Brain, Cortex, Cerebellum, Skeletal muscle, B cells, Temporal lobe

Animal Models for Neuronal Migration Disorders or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
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Publications for Neuronal Migration Disorders

Sources:
51PubMed
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Articles related to Neuronal Migration Disorders:

(show top 50)    (show all 65)
idTitleAuthorsYear
1
Embryonic disruption of the candidate dyslexia susceptibility gene homolog Kiaa0319-like results in neuronal migration disorders. (23831424)
2013
2
Neuronal migration disorders in microcephalic osteodysplastic primordial dwarfism type I/III. (20857301)
2011
3
Molecular genetics of neuronal migration disorders. (21222180)
2011
4
Does subtle disturbance of neuronal migration contribute to schizophrenia and other neurodevelopmental disorders? Potential genetic mechanisms with possible treatment implications. (20207112)
2010
5
Neuronal migration disorders. (19245832)
2010
6
New trends in neuronal migration disorders. (19264520)
2010
7
Experimental neuronal migration disorders following the administration of ibotenate in hamsters: the role of the subventricular zone in the development of cortical dysplasia. (19258286)
2009
8
Neuronal migration disorders: clinical, neuroradiologic and genetics aspects. (19120042)
2009
9
Neuronal migration disorders, agenesis of corpus callosum, preauricular skin tag and bilateral morning glory syndrome in a term newborn infant. (18388784)
2008
10
N-cofilin is associated with neuronal migration disorders and cell cycle control in the cerebral cortex. (17875668)
2007
11
Grey matter heterotopia: what EEG-fMRI can tell us about epileptogenicity of neuronal migration disorders. (16339793)
2006
12
Neuronal migration in developmental disorders. (15921227)
2005
13
Neuronal migration disorders. (16417844)
2005
14
High molecular weight microtubule-associated protein 2 over-expression in neuronal migration disorders caused by N-methyl-D-aspartate receptor activation in hamsters. (15008898)
2004
15
Upregulation of glutamate receptors in rat cerebral cortex with neuronal migration disorders. (15201510)
2004
16
Various findings in surgically treated epilepsy patients with dysembryoplastic neuroepithelial tumors in comparison with those of patients with other low-grade brain tumors and other neuronal migration disorders. (12423388)
2002
17
Pathogenic significance of neuronal migration disorders in temporal lobe epilepsy. (11431720)
2001
18
Lactate and other metabolic changes in neuronal migration disorders. (11240607)
2001
19
Clinical, biochemical and genetic aspects and neuronal migration in peroxisome biogenesis disorders. (11405337)
2001
20
Immunohistochemical expression of doublecortin in the human cerebrum: comparison of normal development and neuronal migration disorders. (10773210)
2000
21
Single cause, polymorphic neuronal migration disorders: an animal model. (11085292)
2000
22
Neuronal migration disorders: from genetic diseases to developmental mechanisms. (10906798)
2000
23
Neuronal migration disorders in man. (11151046)
2000
24
Electrophysiological responses in vivo of hippocampal CA1 pyramidal neurons in an animal model of neuronal migration disorders. (10575261)
1999
25
The right neuron at the wrong place: biology of heterotopic neurons in cortical neuronal migration disorders, with special reference to associated pathologies. (10487203)
1999
26
Neuronal migration disorders in humans and in mouse models--an overview. (10515161)
1999
27
Metabolic changes in neuronal migration disorders: evaluation by combined MRI and proton MR spectroscopy. (10403210)
1999
28
Neuronal migration disorders in cerebral palsy. (19519643)
1999
29
Characterization of neuronal migration disorders in neocortical structures: extracellular in vitro recordings. (9786203)
1998
30
Characterization of neuronal migration disorders in neocortical structures: quantitative receptor autoradiography of ionotropic glutamate, GABA(A) and GABA(B) receptors. (9786204)
1998
31
Neuronal migration disorders. Part I: Terminology, classification, pathophysiology, EEG and epilepsy. (10028855)
1998
32
Neuronal migration disorders. Part II: Magnetic resonance imaging. (10028856)
1998
33
Congenital muscular dystrophy, white-matter abnormalities, and neuronal migration disorders: the expanding concept. (9796753)
1998
34
Transplacentally induced neuronal migration disorders: an animal model for the study of the epilepsies. (9514201)
1998
35
Neuronal migration disorders: heterotopic neocortical neurons in CA1 provide a bridge between the hippocampus and the neocortex. (9707635)
1998
36
Characterization of neuronal migration disorders in neocortical structures. II. Intracellular in vitro recordings. (9658031)
1998
37
Increased seizure susceptibility in adult rats with neuronal migration disorders. (9449431)
1997
38
Ubiquitin-immunoreactive granular inclusions in neuronal migration disorders. (9144592)
1997
39
Mechanisms underlying neuronal migration disorders and epilepsy. (9146989)
1997
40
Neuronal migration disorders presenting with mild clinical symptoms. (8703229)
1996
41
Characterization of neuronal migration disorders in neocortical structures: I. Expression of epileptiform activity in an animal model. (8985688)
1996
42
Neuronal migration disorders and epilepsy: a morphological analysis of three surgically treated patients. (8985686)
1996
43
Neuronal migration disorders increase susceptibility to hyperthermia-induced seizures in developing rats. (8814104)
1996
44
Neuronal migration disorders. (7501186)
1995
45
Electrophysiology of CA1 pyramidal neurons in an animal model of neuronal migration disorders: prenatal methylazoxymethanol treatment. (8777901)
1995
46
Prenatal events and genetic factors in epileptic patients with neuronal migration disorders. (7925168)
1994
47
Neuronal migration disorders: positron emission tomography correlations. (8122881)
1994
48
Stages and patterns of centrifugal arrest of diffuse neuronal migration disorders. (8335148)
1993
49
Epilepsia partialis continua and other seizures arising from the precentral gyrus: high incidence in patients with Rasmussen syndrome and neuronal migration disorders. (1456391)
1992
50
Focal neuronal migration disorders and intractable partial epilepsy: a study of 30 patients. (1789691)
1991

Genetic Variations for Neuronal Migration Disorders

Expression for genes affiliated with Neuronal Migration Disorders

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Neuronal Migration Disorders

Search GEO for disease gene expression data for Neuronal Migration Disorders.

Pathways for genes affiliated with Neuronal Migration Disorders

Sources:
38NCBI BioSystems Database, 4Cell Signaling Technology
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Pathways related to Neuronal Migration Disorders according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.3PAFAH1B1, YWHAE, RELN, DCX
29.2PAFAH1B1, MAP2, DCX, CALB1

Compounds for genes affiliated with Neuronal Migration Disorders

Sources:
45Novoseek, 29IUPHAR, 50PharmGKB, 11DrugBank, 24HMDB
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Compounds related to Neuronal Migration Disorders according to GeneCards/GeneDecks:

(show all 16)
idCompoundScoreTop Affiliating Genes
1nmda45 2910.9MAP2, PVALB
2cyanine459.5CALB2, PVALB
3bromodeoxyuridine459.4CALB1, NES, DCX, MAP2
4methamphetamine45 50 1111.3PVALB, CALB1, MAP2
5quinolinic acid45 2410.1PVALB, CALB2, CALB1
6kainate45 2910.1PVALB, CALB1, NES, DCX, MAP2
7silver458.8PVALB, CALB2, CALB1, MAP2
8paraffin458.8MAP2, NES, CALB2, PVALB
9choline45 11 2410.6PVALB, CALB2, CALB1, NES, MAP2
10acetylcholine45 50 29 11 2412.5PVALB, CALB2, CALB1, NES
11dopamine45 29 11 2411.4PVALB, CALB2, CALB1, FLNA, NES, MAP2
12estrogen458.0PVALB, CALB2, CALB1, NES, MAP2, PAFAH1B1
13glutamate458.0PVALB, CALB2, CALB1, NES, MAP2, RELN
14gaba457.9RELN, PVALB, CALB2, CALB1, ARX, ARFGEF2
15tyrosine457.4PVALB, CALB2, CALB1, FLNA, NES, MAP2
16calcium45 50 11 2410.2PVALB, CALB2, CALB1, FLNA, DCX, DCLK1

GO Terms for genes affiliated with Neuronal Migration Disorders

Sources:
16Gene Ontology
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Cellular components related to Neuronal Migration Disorders according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1microtubule associated complexGO:0058759.7PAFAH1B1, MAP2, DCX

Biological processes related to Neuronal Migration Disorders according to GeneCards/GeneDecks:

(show all 14)
idNameGO IDScoreTop Affiliating Genes
1cell proliferation in forebrainGO:02184610.2ARX, EMX2
2cerebral cortex tangential migrationGO:02180010.2ARX, RELN
3layer formation in cerebral cortexGO:02181910.1PAFAH1B1, GPR56
4central nervous system projection neuron axonogenesisGO:02195210.1DCLK1, DCX
5axon extensionGO:0486759.9DCLK1, DCX
6dendrite morphogenesisGO:0488139.9MAP2, DCLK1, DCX
7negative regulation of JNK cascadeGO:0463299.9PAFAH1B1, FKTN
8hippocampus developmentGO:0217669.7RELN, YWHAE, PAFAH1B1
9brain developmentGO:0074209.6NES, DCX, RELN, GPR56
10cellular response to organic substanceGO:0713109.6MAP2, CALB1
11central nervous system developmentGO:0074179.6NES, DCX, DCLK1, RELN
12intracellular signal transductionGO:0355569.5ARFGEF2, DCX, DCLK1, YWHAE
13G2/M transition of mitotic cell cycleGO:0000869.3NES, YWHAE, PAFAH1B1
14neuron migrationGO:0017648.9PEX2, PAFAH1B1, YWHAE, RELN, DCLK1, DCX

Products for genes affiliated with Neuronal Migration Disorders

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Sources for Neuronal Migration Disorders

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet