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2016 ; 1863
(5
): 934-55
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Peroxisomes in brain development and function
#MMPMID26686055
Berger J
; Dorninger F
; Forss-Petter S
; Kunze M
Biochim Biophys Acta
2016[May]; 1863
(5
): 934-55
PMID26686055
show ga
Peroxisomes contain numerous enzymatic activities that are important for
mammalian physiology. Patients lacking either all peroxisomal functions or a
single enzyme or transporter function typically develop severe neurological
deficits, which originate from aberrant development of the brain, demyelination
and loss of axonal integrity, neuroinflammation or other neurodegenerative
processes. Whilst correlating peroxisomal properties with a compilation of
pathologies observed in human patients and mouse models lacking all or individual
peroxisomal functions, we discuss the importance of peroxisomal metabolites and
tissue- and cell type-specific contributions to the observed brain pathologies.
This enables us to deconstruct the local and systemic contribution of individual
metabolic pathways to specific brain functions. We also review the recently
discovered variability of pathological symptoms in cases with unexpectedly mild
presentation of peroxisome biogenesis disorders. Finally, we explore the emerging
evidence linking peroxisomes to more common neurological disorders such as
Alzheimer's disease, autism and amyotrophic lateral sclerosis.
|ATPases Associated with Diverse Cellular Activities
[MESH]
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