Page last updated: 2024-09-27

Agyria

A smooth brain malformation of the CEREBRAL CORTEX resulting from the abnormal location of developing neurons during corticogenesis. It is characterized by an absence of normal convoluted indentations on the surface of the brain (agyria), or fewer and shallower indentations (pachygryia). There is a reduced number of cortical layers, typically 4 instead of 6, resulting in a thickened cortex, and reduced cerebral white matter that is a reversal of the normal ratio of cerebral white matter to cortex.

Synonyms(7)

Synonym
Lissencephaly
Broad Gyri of Cerebrum
Large Gyri of Cerebrum
Lissencephalia
Pachygyria
Macrogyria
Agyria

Research Excerpts

Overview

ExcerptReference
"Lissencephaly is a devastating neurological disorder caused by defective neuronal migration."( Hirotsune, S; Kengaku, M; Miyakawa, T; Mori, D; Sato, M; Sorimachi, H; Takao, K; Takitoh, T; Umeshima, H; Wynshaw-Boris, A; Yamada, M; Yoshida, Y, 2009)
"Lissencephaly is a severe brain developmental disease in human infants, which is usually caused by mutations in either of two genes, LIS1 and DCX."( Gräf, R; Kuhnert, O; Meyer, I, 2011)
"Lissencephaly is a rare brain malformation characterized by a smooth cerebral surface, thickened cortical mantle and microscopic evidence of incomplete neuronal migration."( Kumar, S; Marwaha, RK; Panigrahi, I; Suthar, R, 2013)

Research

Studies (21)

TimeframeStudies, This Condition (%)All Conditions %
pre-19900 (0.00)23.3326
1990's0 (0.00)12.5806
2000's4 (19.05)18.1394
2010's14 (66.67)28.8240
2020's3 (14.29)9.53
DrugIndicatedRelationship StrengthStudiesTrials
gamma-aminobutyric acid0low10
carbamates0low10
apnea0low10
phenobarbital0low10
potassium chloride0low10
sevoflurane0low10
thyroxine0low10
leucine0low10
valine0low10
fludrocortisone0low10
glutamic acid0low10
aloxistatin0low10
fluorodeoxyglucose f180low20
1-((3,5-dichloro)-2,6-dihydroxy-4-methoxyphenyl)-1-hexanone0low10
leupeptins0low20
acetylleucyl-leucyl-norleucinal0low20
tretinoin0low10
methylazoxymethanol acetate0low10
snj-19450low10
sepharose0low10
calpain0low30
acyclovir0low10
guanosine triphosphate0low10
valacyclovir0low10

Protein Targets (392)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
Chain A, Cruzipain2002
TDP1 protein100010
nuclear receptor ROR-gamma isoform 12002
survival motor neuron protein isoform d2002
Calpain small subunit 10101
Calpain-1 catalytic subunit0303
Cathepsin B0202
ATP-dependent translocase ABCB10001
Replicase polyprotein 1ab0101
Replicase polyprotein 1ab0101
Calpain-1 catalytic subunit0101
Inositol monophosphatase 11001
Chain E, Purine nucleoside phosphorylase0101
Chain A, HADH2 protein1001
Chain B, HADH2 protein1001
Chain A, ATP-DEPENDENT DNA HELICASE Q12002
Chain A, Ferritin light chain3003
Thymidine kinase 0101
thioredoxin reductase6006
phosphopantetheinyl transferase4004
GLS protein3003
Smad32002
thyroid stimulating hormone receptor2002
EWS/FLI fusion protein3003
arylsulfatase A3003
euchromatic histone-lysine N-methyltransferase 27007
activating transcription factor 62002
Histone H2A.x1001
Bloom syndrome protein isoform 14004
hemoglobin subunit beta1001
atrial natriuretic peptide receptor 1 precursor1001
ubiquitin carboxyl-terminal hydrolase 2 isoform a1001
lamin isoform A-delta105005
Solute carrier family 22 member 1 0001
ATP-binding cassette sub-family C member 30202
Multidrug resistance-associated protein 40202
Solute carrier family 22 member 60001
Bile salt export pump0101
Bile salt export pump0303
Purine nucleoside phosphorylase0101
Thymidine kinase, cytosolic0001
POU domain, class 2, transcription factor 20001
Cytochrome P450 2C9 0112
Cytochrome P450 2J20101
Disintegrin and metalloproteinase domain-containing protein 171001
Purine nucleoside phosphorylase0011
Solute carrier family 22 member 60001
Thymidine kinase 0101
Solute carrier family 22 member 80002
Canalicular multispecific organic anion transporter 10202
Thymidine kinase0101
calpain II, partial0101
ClpP1001
PPM1D protein1001
thioredoxin glutathione reductase3003
geminin4004
Papain0101
Interferon beta4004
Procathepsin L0101
Cathepsin G0101
Cathepsin G0101
Guanine nucleotide-binding protein G1001
Neutrophil elastase0101
Myeloblastin0101
ATAD5 protein, partial2002
Microtubule-associated protein tau2002
estrogen-related nuclear receptor alpha3003
D(1A) dopamine receptor2002
neuropeptide S receptor isoform A2002
GALC protein1001
AR protein4004
progesterone receptor1001
glucocorticoid receptor [Homo sapiens]2002
estrogen nuclear receptor alpha4004
aryl hydrocarbon receptor3003
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a3003
nuclear receptor subfamily 1, group I, member 21001
Gamma-aminobutyric acid 0011
Gamma-aminobutyric acid receptor subunit rho-30011
Hsf1 protein0011
regulator of G-protein signaling 43003
bromodomain adjacent to zinc finger domain 2B2002
thyroid hormone receptor beta isoform a3003
transcriptional regulator ERG isoform 31001
histone-lysine N-methyltransferase 2A isoform 2 precursor1001
peripheral myelin protein 222002
histone acetyltransferase KAT2A isoform 13003
Gamma-aminobutyric acid receptor subunit pi0101
Gamma-aminobutyric acid receptor subunit pi1203
Gamma-aminobutyric acid receptor subunit delta0101
Histone deacetylase 30101
Gamma-aminobutyric acid type B receptor subunit 20011
Gamma-aminobutyric acid type B receptor subunit 20101
Gamma-aminobutyric acid receptor subunit alpha-10112
Gamma-aminobutyric acid receptor subunit beta-11203
Gamma-aminobutyric acid receptor subunit alpha-60011
Gamma-aminobutyric acid receptor subunit beta-10112
Gamma-aminobutyric acid receptor subunit delta1203
Gamma-aminobutyric acid receptor subunit gamma-20112
Gamma-aminobutyric acid receptor subunit gamma-21214
Gamma-aminobutyric acid receptor subunit alpha-51203
Gamma-aminobutyric acid receptor subunit alpha-31203
Gamma-aminobutyric acid receptor subunit gamma-20011
Gamma-aminobutyric acid receptor subunit delta0011
Gamma-aminobutyric acid receptor subunit gamma-11203
Gamma-aminobutyric acid receptor subunit alpha-21203
Sodium- and chloride-dependent GABA transporter 10101
Gamma-aminobutyric acid receptor subunit rho-10022
Gamma-aminobutyric acid receptor subunit alpha-20011
Gamma-aminobutyric acid receptor subunit alpha-30011
Gamma-aminobutyric acid receptor subunit gamma-30011
Gamma-aminobutyric acid receptor subunit alpha-41203
Gamma-aminobutyric acid receptor subunit beta-30112
Gamma-aminobutyric acid receptor subunit gamma-31203
Gamma-aminobutyric acid receptor subunit rho-20011
Gamma-aminobutyric acid receptor subunit alpha-61203
Sodium- and chloride-dependent GABA transporter 10101
Sodium- and chloride-dependent taurine transporter0101
Gamma-aminobutyric acid receptor subunit alpha-50112
Sodium- and chloride-dependent GABA transporter 20101
Sodium- and chloride-dependent GABA transporter 30101
Sodium- and chloride-dependent GABA transporter 10101
Sodium- and chloride-dependent GABA transporter 20101
Sodium- and chloride-dependent GABA transporter 30101
Gamma-aminobutyric acid receptor subunit alpha-30112
Gamma-aminobutyric acid receptor subunit alpha-20112
Gamma-aminobutyric acid receptor subunit beta-20112
Sodium- and chloride-dependent betaine transporter0101
Sodium- and chloride-dependent betaine transporter0101
Sodium- and chloride-dependent GABA transporter 30101
Gamma-aminobutyric acid receptor subunit alpha-40112
Gamma-aminobutyric acid receptor subunit beta-10011
Histone deacetylase 40101
Gamma-aminobutyric acid receptor subunit alpha-10011
Gamma-aminobutyric acid receptor subunit alpha-11214
Gamma-aminobutyric acid receptor subunit beta-31203
Gamma-aminobutyric acid receptor subunit beta-30011
Gamma-aminobutyric acid receptor subunit beta-21214
Gamma-aminobutyric acid receptor subunit epsilon0101
4-aminobutyrate aminotransferase, mitochondrial0001
4-aminobutyrate aminotransferase, mitochondrial0001
TAR DNA-binding protein 431001
Histone deacetylase 10101
Gamma-aminobutyric acid receptor subunit alpha-60112
Sterol O-acyltransferase 10011
Proton-coupled amino acid transporter 10203
Gamma-aminobutyric acid receptor subunit alpha-50011
Gamma-aminobutyric acid receptor subunit gamma-10101
Gamma-aminobutyric acid receptor subunit pi0011
Histone deacetylase 70101
GABA theta subunit1203
Histone deacetylase 20101
Polyamine deacetylase HDAC100101
Histone deacetylase 11 0101
Gamma-aminobutyric acid receptor subunit gamma-30101
Histone deacetylase 80101
Gamma-aminobutyric acid receptor subunit alpha-40011
Gamma-aminobutyric acid receptor subunit epsilon1203
Platelet glycoprotein VI0011
Gamma-aminobutyric acid receptor subunit theta0011
Sodium- and chloride-dependent GABA transporter 20101
Gamma-aminobutyric acid receptor subunit gamma-10011
Histone deacetylase 60101
Gamma-aminobutyric acid type B receptor subunit 10011
Histone deacetylase 90101
Gamma-aminobutyric acid receptor subunit theta0101
Histone deacetylase 50101
Gamma-aminobutyric acid type B receptor subunit 10101
Chain A, GLUTAMATE RECEPTOR SUBUNIT 20101
Chain A, Glutamate Receptor Subunit 20101
Chain B, Glutamate Receptor Subunit 20101
Chain A, Slr1257 protein0011
Chain A, Glucosamine--fructose-6-phosphate aminotransferase [isomerizing]0101
glp-1 receptor, partial1001
beta-2 adrenergic receptor1001
peptidyl-prolyl cis-trans isomerase NIMA-interacting 12002
Metabotropic glutamate receptor 80202
Metabotropic glutamate receptor 60113
Glutamate receptor ionotropic, NMDA 2D0101
Excitatory amino acid transporter 40101
Glutamate receptor ionotropic, NMDA 3B0101
Glutathione reductase, mitochondrial0202
Bifunctional aspartokinase/homoserine dehydrogenase 10101
Prothrombin0011
Carbonic anhydrase 10102
Carbonic anhydrase 20102
Integrin beta-30011
Integrin alpha-IIb0011
ATP-citrate synthase 0101
Glutamate receptor 10213
Glutamate receptor 21214
Glutamate receptor 30213
Glutamate receptor 40213
Glutamate receptor ionotropic, kainate 10213
Metabotropic glutamate receptor 10112
Metabotropic glutamate receptor 20011
Metabotropic glutamate receptor 30011
Metabotropic glutamate receptor 40112
Metabotropic glutamate receptor 50011
Metabotropic glutamate receptor 60011
Metabotropic glutamate receptor 70011
Glutamate receptor ionotropic, NMDA 1 0213
Glutamate receptor ionotropic, kainate 10112
Metabotropic glutamate receptor 50113
Glutamate receptor ionotropic, kainate 20213
Glutamate receptor 10213
Glutamate receptor 20213
Glutamate receptor 30112
Glutamate receptor ionotropic, kainate 30202
Excitatory amino acid transporter 10203
Excitatory amino acid transporter 20203
Excitatory amino acid transporter 30203
Metabotropic glutamate receptor 80011
Glutamate receptor 40213
Excitatory amino acid transporter 3 0001
Glutamate racemase0001
Metabotropic glutamate receptor 80112
Fatty-acid amide hydrolase 10101
Glutamate receptor ionotropic, NMDA 2A 0213
Glutamate receptor ionotropic, NMDA 2B0213
Glutamate receptor ionotropic, NMDA 2C0213
Glutamate receptor ionotropic, kainate 40202
Glutamate carboxypeptidase 20101
Glutamate receptor ionotropic, NMDA 10101
Glutamate receptor ionotropic, NMDA 2A0101
Glutamate receptor ionotropic, kainate 20112
Glutamate receptor ionotropic, kainate 30101
Glutamate receptor ionotropic, NMDA 2B0101
Metabotropic glutamate receptor 10113
Metabotropic glutamate receptor 20113
Metabotropic glutamate receptor 70113
Metabotropic glutamate receptor 30113
Metabotropic glutamate receptor 40113
Glutamate receptor ionotropic, NMDA 2C0101
Glutamate receptor ionotropic, kainate 50101
Carbonic anhydrase-like protein, putative0001
Glutamate receptor ionotropic, NMDA 2D0213
Glutamate receptor ionotropic, kainate 50202
Glutamate racemase0001
Prolyl 4-hydroxylase0101
Glutamate receptor ionotropic, NMDA 3A0101
Glutamate receptor ionotropic, NMDA 3B0213
Alpha-ketoglutarate-dependent dioxygenase FTO0101
Glutamate receptor ionotropic, NMDA 3A0213
Chain A, Uracil Phosphoribosyltransferase0011
Chain B, Uracil Phosphoribosyltransferase0011
Chain C, Uracil Phosphoribosyltransferase0011
67.9K protein1001
2-dehydropantoate 2-reductase0011
Ras-related protein Rab-7a0011
4-galactosyl-N-acetylglucosaminide 3-alpha-L-fucosyltransferase 90202
Chain A, AMINOPEPTIDASE0101
Chain A, AMINOPEPTIDASE0101
Chain A, Leucine Aminopeptidase0101
Alkaline phosphatase, tissue-nonspecific isozyme0101
Intestinal-type alkaline phosphatase0101
Large neutral amino acids transporter small subunit 10202
Phospholipase A-2-activating protein0101
Sodium channel protein type 1 subunit alpha0101
Potassium voltage-gated channel subfamily H member 20101
Sodium channel protein type 2 subunit alpha0101
Sodium channel protein type 3 subunit alpha0101
Carbonic anhydrase 40101
Carbonic anhydrase 5A, mitochondrial0101
Taste receptor type 2 member 310011
Taste receptor type 2 member 600011
Carbonic anhydrase 90101
Sodium/potassium/calcium exchanger 40112
Taste receptor type 2 member 140011
Taste receptor type 2 member 130011
Taste receptor type 2 member 90011
Taste receptor type 2 member 40011
Sodium/potassium/calcium exchanger 20112
Spike glycoprotein1001
Calpain-2 catalytic subunit0101
Chain X, Thyroid hormone receptor beta-10011
Chain X, Thyroid hormone receptor beta-10011
RAR-related orphan receptor gamma2002
USP1 protein, partial2002
apical membrane antigen 1, AMA11001
nuclear receptor subfamily 1, group I, member 32002
cytochrome P450 family 3 subfamily A polypeptide 43003
pregnane X nuclear receptor2002
G2002
peroxisome proliferator-activated receptor delta2002
peroxisome proliferator activated receptor gamma2002
v-jun sarcoma virus 17 oncogene homolog (avian)1001
thyroid hormone receptor beta isoform 22002
serine/threonine-protein kinase PLK11001
nuclear factor erythroid 2-related factor 2 isoform 12002
DNA polymerase eta isoform 11001
DNA polymerase kappa isoform 12002
Solute carrier organic anion transporter family member 1A40001
Nuclear receptor subfamily 1 group I member 20011
Solute carrier organic anion transporter family member 1A50001
Rap guanine nucleotide exchange factor 31001
HLA class I histocompatibility antigen, B alpha chain 2002
Transthyretin0102
Cellular tumor antigen p532002
Thyroid hormone receptor alpha0011
Thyroid hormone receptor beta0011
Proliferating cell nuclear antigen0101
Retinoic acid receptor RXR-alpha0246
UDP-glucuronosyltransferase 1A1 0101
Peroxisome proliferator-activated receptor gamma0022
Malate dehydrogenase, mitochondrial0101
Solute carrier organic anion transporter family member 1A20001
Solute carrier organic anion transporter family member 1A30001
Solute carrier organic anion transporter family member 4C10101
Monocarboxylate transporter 80001
Inositol hexakisphosphate kinase 12002
Bile acid receptor0101
Solute carrier organic anion transporter family member 1C10001
Solute carrier organic anion transporter family member 1B10001
cytochrome P450 2C9, partial2002
Chain A, Nuclear Receptor ROR-beta0101
Chain A, Beta-lactamase2002
Chain A, 2-oxoglutarate Oxygenase1001
Luciferase1001
pregnane X receptor1001
hypoxia-inducible factor 1 alpha subunit1001
GLI family zinc finger 31001
aldehyde dehydrogenase 1 family, member A11001
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)1001
estrogen receptor 2 (ER beta)1001
nonstructural protein 11001
retinoic acid nuclear receptor alpha variant 11001
retinoid X nuclear receptor alpha1001
farnesoid X nuclear receptor1001
Parkin1001
vitamin D (1,25- dihydroxyvitamin D3) receptor1001
thyroid stimulating hormone receptor1001
thyrotropin-releasing hormone receptor1001
Caspase-71001
vitamin D3 receptor isoform VDRA1001
chromobox protein homolog 11001
nuclear factor erythroid 2-related factor 2 isoform 21001
caspase-31001
heat shock protein beta-11001
cytochrome P450 3A4 isoform 11001
M-phase phosphoprotein 81001
Voltage-dependent calcium channel gamma-2 subunit1001
Ornithine decarboxylase0001
Amyloid-beta precursor protein0101
Cytochrome P450 1A20011
Cytochrome P450 3A40011
Ornithine decarboxylase0001
60 kDa chaperonin0101
Adenosine receptor A30202
Retinoic acid receptor alpha0227
Cytochrome P450 2D60011
60 kDa heat shock protein, mitochondrial0101
Retinoic acid receptor beta0227
Retinoic acid receptor alpha0123
Retinoic acid receptor gamma 0227
Alpha-1B adrenergic receptor0202
Alpha-1A adrenergic receptor0101
Retinoic acid receptor gamma0123
5-hydroxytryptamine receptor 1A0101
Retinoic acid receptor beta0123
Cellular retinoic acid-binding protein 20011
Alpha-1D adrenergic receptor0101
Mitogen-activated protein kinase 10101
Retinoic acid receptor RXR-alpha0123
Retinoic acid receptor RXR-beta0224
Retinoic acid receptor RXR-beta0123
Retinoic acid receptor RXR-gamma0123
Indoleamine 2,3-dioxygenase 10101
Cytochrome P450 2C190011
Nuclear receptor ROR-alpha0101
Alpha-synuclein0101
Cellular retinoic acid-binding protein 10102
5-hydroxytryptamine receptor 2B0202
Alpha-1A adrenergic receptor0202
Retinoic acid receptor RXR-gamma0224
Nuclear receptor ROR-gamma0112
10 kDa heat shock protein, mitochondrial0101
Cellular retinoic acid-binding protein 10011
Peptidyl-prolyl cis-trans isomerase NIMA-interacting 10213
Thiosulfate sulfurtransferase0101
60 kDa chaperonin 0101
10 kDa chaperonin 0101
Indoleamine 2,3-dioxygenase 20101
Nuclear receptor ROR-beta0101
ATPase family AAA domain-containing protein 51001
Ataxin-21001
ATP-dependent phosphofructokinase1001
Solute carrier family 15 member 10203
Solute carrier family 15 member 10101
Solute carrier family 15 member 20101
Valacyclovir hydrolase0001
Chain A, Arginase 10101
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]