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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

ABSTRACT: Fatty acid 2-hydroxylase (FA2H) is responsible for the synthesis of 2-hydroxylated fatty acids, which are a building block of sphingolipids. This 2-hydroxylated sphingolipids can be found in various tissues such as brain, spinal cord, skin, testis, ovary, kidney, stomach, or intestine with a notably high occurrence in the insulating myelin sheath of neurons. Mutations in the FA2H gene can cause the neurodegenerative disease spastic paraplegia 35 (SPG35), which is also known as fatty acid hydroxylase-associated neurodegeneration. Moreover, FA2H knock-out mice largely resemble the SPG35 human disease phenotype. To further elucidate the connection between FA2H and SPG35, we performed a comparative quantitative proteome analysis of peripheral nervous system myelin of wildtype and FA2H-KO mice at different ages (6, 13, and 17 months) using isobaric labeling with tandem mass tags (TMT).

INSTRUMENT(S): LTQ Orbitrap Velos

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Myelin, Sciatic Nerve

SUBMITTER: Robert Hardt

LAB HEAD: Matthias Eckhardt

PROVIDER: PXD030244 | Pride | 2022-04-22

REPOSITORIES: Pride

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			Action	DRS
	140110_NM-RG_A01.raw	Raw
	140110_NM-RG_A02.raw	Raw
	140110_NM-RG_A03.raw	Raw
	140110_NM-RG_A04.raw	Raw
	140110_NM-RG_A05.raw	Raw

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Altered turnover of the CNS myelin protein Opalin in a mouse model of hereditary spastic paraplegia 35

Project description:Fatty acid 2-hydroxylase (FA2H) is responsible for the synthesis of 2-hydroxylated fatty acids, which are a building block of sphingolipids. This 2-hydroxylated sphingolipids can be found in various tissues such as brain, spinal cord, skin, testis, ovary, kidney, stomach, or intestine with a notably high occurrence in the insulating myelin sheath of neurons. Mutations in the FA2H gene can cause the neurodegenerative disease spastic paraplegia 35 (SPG35), which is also known as fatty acid hydroxylase-associated neurodegeneration. Moreover, FA2H knock-out mice largely resemble the SPG35 human disease phenotype. To further elucidate the connection between FA2H and SPG35, we performed a comparative quantitative proteome analysis of central nervous system myelin of wildtype and FA2H-KO mice at different ages (6, 13, and 17 months) using isobaric labeling with tandem mass tags (TMT).

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