Article

Establishment of spinocerebellar ataxia type 34 model mice accompanied by early glial activation and degeneration of cerebellar neurons

2025-10-16

Abstract excerpt

Spinocerebellar ataxia type 34 (SCA34) is an autosomal dominant neurodegenerative disease primarily characterized by progressive cerebellar atrophy and ataxia, frequently accompanied by cognitive dysfunction and erythrokeratodermia variabilis. In 2014, missense mutations in the gene encoding elongation of very long chain fatty acids protein 4 (ELOVL4) were identified as the causative gene for SCA34. ELOVL4, which...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: bdda7644-1161-51c4-8501-f838c7c7dc74
DOI: 10.1101/2025.10.15.682296

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Establishment of spinocerebellar ataxia type 34 model mice accompanied by early glial activation and degeneration of cerebellar neuronsDOI 10.1101/2025.10.15.682296Read

W246G Mutant ELOVL4 Impairs Synaptic Plasticity in Parallel and Climbing Fibers and Causes Motor Defects in a Rat Model of SCA34.Semantic proximity Read

The Spinocerebellar Ataxia 34-Causing W246G ELOVL4 Mutation Does Not Alter Cerebellar Neuron Populations in a Rat Model.Semantic proximity Read

W246G Mutant ELOVL4 Impairs Synaptic Plasticity in Parallel and Climbing Fibers and Causes Motor Defects in a Rat Model of SCA34Semantic proximity Read

Association of cerebellar inflammation and neurodegeneration in a novel spinocerebellar ataxia type 13 mouse modelSemantic proximity Read

Early Neuroimmune Modulation in Hereditary Cerebellar Ataxias: Experimental Opportunities in Zebrafish ModelsSemantic proximity Read

Cerebellar ataxias: an update.Semantic proximity Read

The Elovl4 Spinocerebellar Ataxia-34 Mutation 736T>G (p.W246G) Impairs Retinal Function in the Absence of Photoreceptor Degeneration.Semantic proximity Read

Glial cell activation precedes neurodegeneration in the cerebellar cortex of the YG8-800 murine model of Friedreich’s ataxiaSemantic proximity Read

Rare mutation in ELOVL4 gene in SCA34 and cognitive affection: Expounding the role of cerebellum.Semantic proximity Read

A Novel Mutation in ELOVL4 Leading to Spinocerebellar Ataxia (SCA) With the Hot Cross Bun Sign but Lacking Erythrokeratodermia: A Broadened Spectrum of SCA34.Semantic proximity Read

Disrupted Calcium Signaling in Animal Models of Human Spinocerebellar Ataxia (SCA)Semantic proximity Read

Loss of intracerebellar heterogeneity and selective vulnerability in Spinocerebellar ataxia type 1 neurodegenerationSemantic proximity Read