LGI4

Protein identityUniProt · HPA

UniProt accession

Q8N135

Gene

LGI4

Ensembl

ENSG00000153902

Chromosome

19

Canonical length

537 aa

Protein class

Disease related genes, Human disease related genes, Predicted intracellular proteins, Predicted secreted proteins

Subcellular location

Golgi apparatus

Secretome location

Secreted in brain

OverviewNCBI Gene

Involved in regulation of myelination. Predicted to be located in extracellular region. Predicted to be active in extracellular space. Implicated in arthrogryposis multiplex congenita-1 and childhood absence epilepsy. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

537 residues, UniProt reviewed canonical sequence.

>Q8N135|LGI4
     1  MGGAGILLLL LAGAGVVVAW RPPKGKCPLR CSCSKDSALC EGSPDLPVSF SPTLLSLSLV
    61  RTGVTQLKAG SFLRIPSLHL LLFTSNSFSV IEDDAFAGLS HLQYLFIEDN EIGSISKNAL
   121  RGLRSLTHLS LANNHLETLP RFLFRGLDTL THVDLRGNPF QCDCRVLWLL QWMPTVNASV
   181  GTGACAGPAS LSHMQLHHLD PKTFKCRAIE LSWFQTVGES ALSVEPFSYQ GEPHIVLAQP
   241  FAGRCLILSW DYSLQRFRPE EELPAASVVS CKPLVLGPSL FVLAARLWGG SQLWARPSPG
   301  LRLAPTQTLA PRRLLRPNDA ELLWLEGQPC FVVADASKAG STTLLCRDGP GFYPHQSLHA
   361  WHRDTDAEAL ELDGRPHLLL ASASQRPVLF HWTGGRFERR TDIPEAEDVY ATRHFQAGGD
   421  VFLCLTRYIG DSMVMRWDGS MFRLLQQLPS RGAHVFQPLL IARDQLAILG SDFAFSQVLR
   481  LEPDKGLLEP LQELGPPALV APRAFAHITM AGRRFLFAAC FKGPTQIYQH HEIDLSA

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against LGI4 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Secreted

Secreted

Yes

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.26

Highest tissue expression

102 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about LGI4.

Disease | AllUniProt

Conditions LGI4 is implicated in, by any mechanism.

• Arthrogryposis multiplex congenita 1, neurogenic, with myelin defect (AMC1) MIM:617468

Disease | GeneticClinVar

19 pathogenic / likely-pathogenic of 158 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Arthrogryposis multiplex congenita 1, neurogenic, with myelin defect
• Arthrogryposis multiplex congenita 2, neurogenic type
• Arthrogryposis multiplex congenita
• Fetal akinesia deformation sequence 1

Disease | ImmuneIEDB

Conditions an epitope on LGI4 was assayed in.

• glioblastoma T cell

ReferencesPubMed · IEDB

Publications for LGI4 from three distinct lines of evidence, kept separate because they answer different questions: whether antibodies are directed at the protein, whether a B-cell epitope has been mapped on it, and whether a T-cell epitope has. Each is labelled with its source.

Sources: PubMed — antigen-level antibody evidence from a custom retrieval. Records matching a controlled set of autoantibody terms (the MeSH descriptors Autoantibodies and Autoantigens, with title and abstract term variants) were obtained through NCBI E-utilities, and their titles and abstracts parsed for constructions that direct an antibody at a named protein rather than for co-occurrence. Captured names were resolved against UniProt nomenclature and each antigen adjudicated individually against the source text. IEDB — curated epitope assays from the Immune Epitope Database (Vita et al., Nucleic Acids Research 2019). Bibliographic records from PubMed and MeSH, U.S. National Library of Medicine; citation metrics from NIH iCite (Hutchins et al., PLoS Biology 2016). Titles link to PubMed; abstracts are not reproduced here. The NLM does not endorse this analysis.

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.68

gnomAD pLI

0.01

gnomAD missense Z

1.07

DepMap mean gene effect

0.09

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 4% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• adult locomotory behavior
• glial cell proliferation
• myelination in peripheral nervous system
• neuron maturation
• regulation of myelination

Cellular components

• extracellular region
• extracellular space

Protein domainsUniProt · Pfam · InterPro

• Cysteine-rich flanking region, C-terminal
• Leucine-rich repeat
• Leucine-rich repeat, typical subtype
• Leucine-rich glioma-inactivated , EPTP repeat
• EAR
• Leucine-rich repeat domain superfamily
• Leucine-rich glioma-inactivated/LGI-related
• EPTP domain
• Leucine rich repeat

KeywordsUniProt

• Glycoprotein
• Leucine-rich repeat
• Repeat
• Secreted
• Signal

InteractionsUniProt · HPA

Protein binding partners of LGI4 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• ADAM11