{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Jordans S"],"funding":["Deutsche Forschungsgemeinschaft","Rheinische Friedrich-Wilhelms-Universität Bonn"],"pagination":["3969-3979"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC9167166"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["59(7)"],"pubmed_abstract":["PNS and CNS myelin contain large amounts of galactocerebroside and sulfatide with 2-hydroxylated fatty acids. The underlying hydroxylation reaction is catalyzed by fatty acid 2-hydroxylase (FA2H). Deficiency in this enzyme causes a complicated hereditary spastic paraplegia, SPG35, which is associated with leukodystrophy. Mass spectrometry-based proteomics of purified myelin isolated from sciatic nerves of Fa2h-deficient (Fa2h<sup>-/-</sup>) mice revealed an increase in the concentration of the three proteins Cadm4, Mpp6 (Pals2), and protein band 4.1G (Epb41l2) in 17-month-old, but not in young (4 to 6-month-old), Fa2h<sup>-/-</sup> mice. These proteins are known to form a complex, together with the protein Lin7, in Schmidt-Lanterman incisures (SLIs). Accordingly, the number of SLIs was sig"],"journal":["Molecular neurobiology"],"pubmed_title":["Age-Dependent Increase in Schmidt-Lanterman Incisures and a Cadm4-Associated Membrane Skeletal Complex in Fatty Acid 2-hydroxylase Deficient Mice: a Mouse Model of Spastic Paraplegia SPG35."],"pmcid":["PMC9167166"],"funding_grant_id":["SFB645 project B5"],"pubmed_authors":["Jordans S","Winter D","Hardt R","Becker I","Wang-Eckhardt L","Eckhardt M"],"additional_accession":[]},"is_claimable":false,"name":"Age-Dependent Increase in Schmidt-Lanterman Incisures and a Cadm4-Associated Membrane Skeletal Complex in Fatty Acid 2-hydroxylase Deficient Mice: a Mouse Model of Spastic Paraplegia SPG35.","description":"PNS and CNS myelin contain large amounts of galactocerebroside and sulfatide with 2-hydroxylated fatty acids. The underlying hydroxylation reaction is catalyzed by fatty acid 2-hydroxylase (FA2H). Deficiency in this enzyme causes a complicated hereditary spastic paraplegia, SPG35, which is associated with leukodystrophy. Mass spectrometry-based proteomics of purified myelin isolated from sciatic nerves of Fa2h-deficient (Fa2h<sup>-/-</sup>) mice revealed an increase in the concentration of the three proteins Cadm4, Mpp6 (Pals2), and protein band 4.1G (Epb41l2) in 17-month-old, but not in young (4 to 6-month-old), Fa2h<sup>-/-</sup> mice. These proteins are known to form a complex, together with the protein Lin7, in Schmidt-Lanterman incisures (SLIs). Accordingly, the number of SLIs was sig","dates":{"release":"2022-01-01T00:00:00Z","publication":"2022 Jul","modification":"2026-05-10T01:38:31.35Z","creation":"2025-02-19T01:12:05.651Z"},"accession":"S-EPMC9167166","cross_references":{"pubmed":["35445918"],"doi":["10.1007/s12035-022-02832-4"]}}