Autism spectrum disorder 1

disease
On this page

Also known as Asperger syndromeautism spectrum disorder without disorder of intellectual development and with mild or no impairment of functional language

Summary

Autism spectrum disorder 1 (MONDO:0100610) is a disease with 6 cohort genes (5 GWAS associations across 2 studies) and 29 clinical trials. Top therapeutic interventions include milnacipran and risperidone.

At a glance

  • Cohort genes: 6
  • GWAS associations: 5
  • Clinical trials: 29

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

FieldValue
Canonical nameautism spectrum disorder 1
Mondo IDMONDO:0100610
MeSHD020817
Orphanet1162
DOIDDOID:0050432
ICD-11366940030
NCITC97159
SNOMED CT23560001
GARD0028001
Is cancer (heuristic)no

Also known as: Asperger syndrome · autism spectrum disorder without disorder of intellectual development and with mild or no impairment of functional language

Data availability: 5 GWAS associations (2 studies).

Disease family

Classification path: disease › human disease › disease by developmental or physiological process › psychiatric disordermental disorderdevelopmental disorder of mental healthpervasive developmental disorderautism spectrum disorderautism spectrum disorder 1

Related subtypes (4): autism, PAX5-related B lymphopenia and autism spectrum disorder, autism spectrum disorder 2, autism spectrum disorder 3

Genetics & variants

GWAS landscape

5 GWAS associations across 2 studies. Top hits map to 5 distinct genes (as reported by GWAS).

Top associations by p-value

rsIDp-valueGeneRisk alleleOdds ratio
rs47031291e-06MTCO1P24 - CTBP2P4?
rs15509762e-06NTM?
rs105108374e-06FHIT?
rs8920555e-06RASGRP4?
rs71794567e-06RNF111, SLTM?

Top studies (by case count)

StudyLead authorYearCasesControlsTitle
GCST003029Warrier V20152940A Pooled Genome-Wide Association Study of Asperger Syndrome.
GCST000904Salyakina D201000Variants in several genomic regions associated with asperger disorder.

Variant details and genetic-evidence tiers

Tier distribution (top 50 variants)

TierVariants
Tier 1: coding1
Tier 2: splice/UTR0
Tier 3: regulatory0
Tier 4: intronic/intergenic4

MAF distribution

BucketVariants
common (>=0.05)5
low_freq (0.01-0.05)0
rare (<0.01)0
unknown0

Functional consequences

ConsequenceCount
intron_variant3
intergenic_variant1
missense_variant1

Top variants

rsIDChrPosAllelesMAFConsequenceGenep-valueTier
rs4703129598553032C>A,G,T0.41intergenic_variantMTCO1P24 - CTBP2P41e-06Tier 4: intronic/intergenic
rs155097611131450176T>A,C0.33intron_variantNTM2e-06Tier 4: intronic/intergenic
rs10510837360304113G>A0.12intron_variantFHIT4e-06Tier 4: intronic/intergenic
rs8920551938422124A>C,G0.33missense_variantRASGRP45e-06Tier 1: coding
rs71794561558882340G>C,T0.39intron_variantRNF111, SLTM7e-06Tier 4: intronic/intergenic

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 0 · Orphanet: 1 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

Orphanet rare-disease linkage (cohort genes)

GeneOrphanet IDRare disease
FHITOrphanet:422526Hereditary clear cell renal cell carcinoma

Cohort genes → proteins

6 cohort genes, 6 distinct canonical proteins.

Evidence partition

SubsetGenes
gwas_only6

Cohort genes (full)

SymbolHGNCEnsemblUniProtNameEvidence
RNF111HGNC:17384ENSG00000157450Q6ZNA4E3 ubiquitin-protein ligase Arkadiagwas
NTMHGNC:17941ENSG00000182667Q9P121Neurotrimingwas
SLTMHGNC:20709ENSG00000137776Q9NWH9SAFB-like transcription modulatorgwas
CIMAP1DHGNC:26841ENSG00000181781Q3SX64Protein CIMAP1Dgwas
RGMBHGNC:26896ENSG00000174136Q6NW40Repulsive guidance molecule Bgwas
FHITHGNC:3701ENSG00000189283P49789Bis(5’-adenosyl)-triphosphatasegwas

Cohort function summary

Lead sentence per gene, UniProt-curated.

SymbolProtein nameFunction (lead sentence)
RNF111E3 ubiquitin-protein ligase ArkadiaE3 ubiquitin-protein ligase.
NTMNeurotriminNeural cell adhesion molecule.
SLTMSAFB-like transcription modulatorWhen overexpressed, acts as a general inhibitor of transcription that eventually leads to apoptosis.
RGMBRepulsive guidance molecule BMember of the repulsive guidance molecule (RGM) family that contributes to the patterning of the developing nervous system.
FHITBis(5’-adenosyl)-triphosphatasePossesses dinucleoside triphosphate hydrolase activity.

Protein-family classification

Druggable: 2 · Difficult: 1 · Unknown: 3 · Druggable fraction: 0.33

Family distribution

Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.

FamilyGenesFoldFDR
Antibody/Immunoglobulin14.9×0.719
Enzyme (other)12.0×0.719
Transcription factor11.4×0.719
Other/Unknown30.9×0.758

Per-gene assignment

SymbolFamilyDruggable?ECInterPro (top 3)
RNF111Transcription factorno2.3.2.27Znf_RING, Znf_RING/FYVE/PHD, RNF111_N
NTMAntibody/ImmunoglobulinyesIg_sub2, Ig_sub, Ig-like_dom
SLTMOther/UnknownnoRRM_dom, SAP_dom, Nucleotide-bd_a/b_plait_sf
CIMAP1DOther/UnknownnoSHIPPO-rpt, CIMAP
RGMBOther/UnknownnoRGM_C, RGM_N, RGM
FHITEnzyme (other)yes3.6.1.29HIT-like, Histidine_triad_CS, HIT-like_sf

Expression context

Cohort genes with no expression data: 0.

5 cohort genes are a single-cell marker in ≥1 SCXA experiment.

Breadth distribution (Bgee present_calls)

BucketGenes
narrow (1-5 tissues)0
moderate (6-20)0
broad (>20)6
unknown0

Top tissues across cohort

TissueCohort genes
endothelial cell1
oocyte1
secondary oocyte1
cerebellar cortex1
cerebellar hemisphere1
right hemisphere of cerebellum1
calcaneal tendon1
sural nerve1
tibia1
left testis1
male germ line stem cell (sensu Vertebrata) in testis1
right testis1
ileal mucosa1
pylorus1
upper arm skin1
left adrenal gland cortex1
right adrenal gland1
right adrenal gland cortex1

Per-gene tissue summary (top 30)

SymbolBgee breadthFANTOM5 breadthSCXATop tissues
RNF111283ubiquitousmarkersecondary oocyte, oocyte, endothelial cell
NTM231ubiquitousmarkerright hemisphere of cerebellum, cerebellar cortex, cerebellar hemisphere
SLTM291ubiquitousmarkercalcaneal tendon, sural nerve, tibia
CIMAP1D134tissue_specificyesright testis, left testis, male germ line stem cell (sensu Vertebrata) in testis
RGMB254ubiquitousmarkerileal mucosa, pylorus, upper arm skin
FHIT188ubiquitousmarkerright adrenal gland, right adrenal gland cortex, left adrenal gland cortex

Protein interactions among cohort

Intra-cohort edges: 0.

Hub genes (top 10 by interactor count)

SymbolInteractor count
SLTM2,598
RNF1112,198
NTM1,777
FHIT1,690
RGMB531
CIMAP1D501

Structural data

PDB: 4 · AlphaFold-only: 2 · No structure: 0

Cohort genes with PDB structures (top 30)

SymbolUniProtPDB entries
RGMBQ6NW4011
FHITP497899
RNF111Q6ZNA44
NTMQ9P1212

AlphaFold-only cohort genes (top 30 by pLDDT)

SymbolUniProtpLDDT
CIMAP1DQ3SX6467.70
SLTMQ9NWH952.38

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 19. Enrichment computed across 6 evidence-associated genes (3 with Reactome annotation).

Pathways by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 3 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

PathwayCohort genesFoldFDRSample cohort genes
Global Genome Nucleotide Excision Repair (GG-NER)1152.3×0.032RNF111
Netrin-1 signaling1146.4×0.032RGMB
Transcriptional activity of SMAD2/SMAD3:SMAD4 heterotrimer1122.8×0.032RNF111
Downregulation of SMAD2/3:SMAD4 transcriptional activity1122.8×0.032RNF111
SMAD2/SMAD3:SMAD4 heterotrimer regulates transcription1102.9×0.032RNF111
Nucleotide Excision Repair195.2×0.032RNF111
Formation of Incision Complex in GG-NER184.6×0.032RNF111
Signaling by TGF-beta Receptor Complex166.8×0.035RNF111
Post-translational modification: synthesis of GPI-anchored proteins156.0×0.037NTM
Signaling by TGFB family members138.5×0.049RNF111
DNA Repair132.8×0.052RNF111
Class I MHC mediated antigen processing & presentation123.4×0.067RNF111
Antigen processing: Ubiquitination & Proteasome degradation112.4×0.115RNF111
Adaptive Immune System19.9×0.132RNF111
RNA Polymerase II Transcription17.5×0.161RNF111
Gene expression (Transcription)16.0×0.189RNF111
Generic Transcription Pathway15.0×0.208RNF111
Immune System14.3×0.226RNF111
Signal Transduction13.4×0.267RNF111

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 5 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO termCohort genesFoldFDRSample cohort genes
diadenosine triphosphate catabolic process13370.4×0.006FHIT
global genome nucleotide-excision repair11685.2×0.006RNF111
purine nucleotide metabolic process11123.5×0.006FHIT
neuron recognition1674.1×0.007NTM
regulation of mRNA processing1177.4×0.021SLTM
cell adhesion215.0×0.021NTM, RGMB
intrinsic apoptotic signaling pathway by p53 class mediator1116.2×0.022FHIT
positive regulation of transforming growth factor beta receptor signaling pathway1105.3×0.022RNF111
pattern specification process193.6×0.022RNF111
negative regulation of proteasomal ubiquitin-dependent protein catabolic process180.2×0.024FHIT
positive regulation of protein ubiquitination142.7×0.039RNF111
BMP signaling pathway140.1×0.039RGMB
protein polyubiquitination123.1×0.062RNF111
ubiquitin-dependent protein catabolic process114.8×0.089RNF111
protein ubiquitination18.3×0.146RNF111
apoptotic process15.7×0.186SLTM
positive regulation of DNA-templated transcription15.6×0.186RGMB
signal transduction13.2×0.290RGMB
regulation of transcription by RNA polymerase II12.3×0.361SLTM

Therapeutics

Drug target analysis

Approved (phase 4): 1 · Phase ≥3: 2 · Phased (≥1): 2 · Undrugged: 4

Druggability breadth: 2 of 6 evidence-associated genes (33%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Genes with an approved drug

The molecule shown is one approved compound that hits the gene — not necessarily a drug of choice or one indicated for this disease.

SymbolExample approved molecule
SLTMCABOZANTINIB

Top cohort targets by molecule count

SymbolMoleculesMax phase
SLTM14
FHIT13
RNF11100
NTM00
CIMAP1D00
RGMB00

Drugs targeting cohort genes (top 30)

MoleculeMax phaseTargets in cohort
CABOZANTINIB4SLTM
SURAMIN3FHIT

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 2.

Cohort genes with ChEMBL bioactivity (full, sorted by assay count)

SymbolAssaysType breakdown
FHIT21Binding:19, ADMET:2
SLTM14Binding:14

Cohort enzymes (BRENDA EC)

SymbolEC numbersNames
RNF1112.3.2.27RING-type E3 ubiquitin transferase
FHIT3.6.1.29bis(5’-adenosyl)-triphosphatase

Pharmacogenomics

Cohort genes with a PharmGKB record: 6; with CPIC/DPWG dosing guidelines: 0.

No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).

Chemical tractability of cohort targets

2 approved/phased compounds have measured bioactivity against a cohort gene (and aren’t yet in disease-level trials). This is a research / tractability signal, NOT a therapeutic recommendation — a bioactivity row often reflects off-target or screening binding (e.g. promiscuous kinase inhibitors against a cohort kinase), implying no disease mechanism.

CompoundMax phaseCohort target (bioactivity)
CABOZANTINIB4SLTM
SURAMIN3FHIT

Druggability pyramid

Cohort genes binned by druggability tier (high → low):

TierDefinitionGenesSymbols
AApproved (phase 4 drug)1SLTM
BPhased (≥1) drug, not yet approved1FHIT
CDruggable family + PDB, no drug1NTM
DDruggable family + AlphaFold only, no drug0
EDifficult family or no structure, no drug3RNF111, CIMAP1D, RGMB

Undrugged target profiles

4 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).

SymbolChEMBL assaysDrugged partners (top 3)
RNF1110
NTM0
CIMAP1D0
RGMB0

Clinical trials & evidence

Clinical trials

Clinical trials: 29.

Phase distribution (across all retrieved trials)

PhaseTrials
Not specified20
PHASE25
PHASE41
PHASE31
PHASE1/PHASE21
PHASE11

Top trials by phase / activity

NCTPhaseStatusTitle
NCT01337700PHASE4COMPLETEDMilnacipran in Autism and the Functional Locus Coeruleus and Noradrenergic Model of Autism
NCT00261508PHASE3COMPLETEDA Study of the Effectiveness and Safety of Risperidone Versus Placebo in the Treatment of Children With Autistic Disorder and Other Pervasive Developmental Disorders (PDD)
NCT00280670PHASE2COMPLETEDCBT for Comorbid Anxiety Disorders in Children With Autism, Asperger Syndrome, or PDD-NOS
NCT00453180PHASE2COMPLETEDA Study of Oral N-Acetylcysteine in Children With Autism Spectrum Disorders
NCT00619190PHASE2COMPLETEDStudy of Aripiprazole to Treat Children and Adolescents With Autism
NCT00725556PHASE1/PHASE2COMPLETEDThe Development of Autistic Children Based on Mothers´Response in Autism Behavior Checklist
NCT01592747PHASE2COMPLETEDWithdrawal Study of Memantine in Pediatric Patients With Autism, Asperger’s Disorder, or Pervasive Developmental Disorder Not Otherwise Specified Previously Treated With Memantine
NCT01731119PHASE2COMPLETEDStudy of Lurasidone in Treating Antipsychotic Naive or Quasi-Naive Children and Adolescents
NCT01187784PHASE1COMPLETEDCognitive-Behavioral Therapy for Children With High-Functioning Autism Spectrum Disorder and Anxiety
NCT04532424Not specifiedRECRUITINGTranscranial Magnetic Stimulation for Restricted and Repetitive Behavior in ASD
NCT04654260Not specifiedACTIVE_NOT_RECRUITINGBehavior Therapy for Irritability in Autism
NCT05176808Not specifiedRECRUITINGTelehealth Parent-Implemented Intervention for Young Children With Autism Spectrum Disorder (ASD)
NCT06187090Not specifiedRECRUITINGThe Supplementation Therapy in Autism and Response to Treatment Study
NCT06227780Not specifiedRECRUITINGAlpha Auditory Entrainment for Cognitive Enhancement and Sensory Hypersensitivity in Youth With Developmental Disorders
NCT06859918Not specifiedRECRUITINGRandomized Clinical Trial of TUNE In 3.0: A Social/Emotional Program for Adults With Autism Spectrum Disorder
NCT00464477Not specifiedCOMPLETEDAdvanced Grandparental Age as a Risk Factor for Autism
NCT00505830Not specifiedUNKNOWNEvaluation of the French Version of Screening Questionnaires for Autism and Asperger Syndrome: AQ, EQ and SQ
NCT00693953Not specifiedWITHDRAWNUsing the Digital EEG Spectral Analysis in Assessing Neuroelectrical Processing Abnormalities in Autism
NCT00848874Not specifiedCOMPLETEDPortable Visual Guidance System Phase II
NCT01655173Not specifiedCOMPLETEDCognitive Behavioural Therapy (CBT) and Recreational Activity for Autism Spectrum Disorders (ASD)
NCT01921244Not specifiedCOMPLETEDShared Decision Making to Improve Care and Outcomes for Children With Autism
NCT02250339Not specifiedCOMPLETEDA Prospective Observational Study of Family-based Interventions for Children With Neuropsychiatric and/or Psychiatric Disorders
NCT02800681Not specifiedCOMPLETEDPsychopathological Differences Between Asperger Syndrome and Schizotypal Disorder in an Adult Sample
NCT03170453Not specifiedCOMPLETEDConfirmatory Efficacy Trial of Cognitive Enhancement Therapy for Adult Autism Spectrum Disorder
NCT03560453Not specifiedCOMPLETEDFacilitating Employment for Youth With Autism
NCT03672266Not specifiedCOMPLETEDStudies of Brain and Body Interaction
NCT04788537Not specifiedCOMPLETEDServices to Enhance Social Functioning in Adults With Autism Spectrum Disorders
NCT05509231Not specifiedUNKNOWNEffects of a Multimodal Exercise Program for Children With ASD
NCT05958680Not specifiedUNKNOWNASDactive: An Integrated Theory-based Intervention to Promote Habitual Physical Activity

Drugs tested across these trials (top 30)

MoleculeMax phaseTrials referencing
MILNACIPRAN41
RISPERIDONE41
CHEMBL543550001
1R,2S-MILNACIPRAN01