Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Insights into retinal disease and non-tubulin glutamylation from a RPGR-TTLL5 complex structure.

The Journal of cell biology - 1 Jun 2026

Park James H, Liu Richard J Y, Sun Xun, Mahalingan Kishore K, Hiriyanna Suja, Li Tiansen, Roll-Mecak Antonina

Abstract excerpt

Mutations in retinitis pigmentosa GTPase regulator (RPGR) cause photoreceptor degeneration, vision loss, and eventual blindness. RPGR function requires glutamylation by tubulin tyrosine ligase-like 5 (TTLL5) whose mutation is also linked to severe forms of retinal degeneration. How TTLL5 targets RPGR and how mutations in either protein cause disease are unknown. Here we report the 2.8-Å X-ray crystal structure of...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Crystallography, X-Ray
Share to Eye Proteins

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Models, Molecular

Share to Mutation

Share to Peptide Synthases

Share to Protein Binding

Share to Tubulin

Source

Open in PubMed Open full text

Identifiers

PMID: 41941273
DOI: 10.1083/jcb.202508020
PMCID: PMC13052805

Genes

RPGR
TTLL5

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Insights into retinal disease and non-tubulin glutamylation from a RPGR-TTLL5 complex structure.PMID 41941273Read ↗

Loss of RPGR glutamylation underlies the pathogenic mechanism of retinal dystrophy caused by TTLL5 mutations.Semantic proximity Read ↗

Biallelic variants in TTLL5, encoding a tubulin glutamylase, cause retinal dystrophy.Semantic proximity Read ↗

Impaired glutamylation of RPGRORF15 underlies the cone-dominated phenotype associated with truncating distal ORF15 variants.Semantic proximity Read ↗

Excessive tubulin glutamylation leads to progressive cone-rod dystrophy and loss of outer segment integrity.Semantic proximity Read ↗

Glutamylation imbalance leads to photoreceptor cell degenerationSemantic proximity Read ↗

Expanding the phenotype of TTLL5-associated retinal dystrophy: a case series.Semantic proximity Read ↗

Mutations in AGBL5, Encoding α-Tubulin Deglutamylase, Are Associated With Autosomal Recessive Retinitis Pigmentosa.Semantic proximity Read ↗

Alterations in the balance of tubulin glycylation and glutamylation in photoreceptors leads to retinal degeneration.Semantic proximity Read ↗

Expression of recombinant human glutamylating TTLLs in human cells leads to differential tubulin glutamylation patterns, with only TTLL6 disrupting microtubule dynamicsSemantic proximity Read ↗

Mining the CRBN Target Space Redefines Rules for Molecular Glue-induced Neosubstrate RecognitionSemantic proximity Read ↗

Structural and Functional Insights into GID/CTLH E3 Ligase ComplexesSemantic proximity Read ↗

Mutations in a BTB-Kelch protein, KLHL7, cause autosomal-dominant retinitis pigmentosa.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.