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Myelination of the nervous system: mechanisms and functions
Myelination of axons in the nervous system of vertebrates enables fast, saltatory impulse
propagation, one of the best-understood concepts in neurophysiology. However, it took a …
propagation, one of the best-understood concepts in neurophysiology. However, it took a …
X‐linked adrenoleukodystrophy: pathology, pathophysiology, diagnostic testing, newborn screening and therapies
Adrenoleukodystrophy (ALD) is a rare X‐linked disease caused by a mutation of the
peroxisomal ABCD1 gene. This review summarizes our current understanding of the …
peroxisomal ABCD1 gene. This review summarizes our current understanding of the …
Pharmacological advances in mitochondrial therapy
Mitochondria play a vital role in cellular metabolism and are central mediator of intracellular
signalling, cell differentiation, morphogenesis and demise. An increasingly higher number of …
signalling, cell differentiation, morphogenesis and demise. An increasingly higher number of …
Redox interplay between mitochondria and peroxisomes
Reduction-oxidation or “redox” reactions are an integral part of a broad range of cellular
processes such as gene expression, energy metabolism, protein import and folding, and …
processes such as gene expression, energy metabolism, protein import and folding, and …
[HTML][HTML] Peroxisomes in brain development and function
J Berger, F Dorninger, S Forss-Petter… - Biochimica Et Biophysica …, 2016 - Elsevier
Peroxisomes contain numerous enzymatic activities that are important for mammalian
physiology. Patients lacking either all peroxisomal functions or a single enzyme or …
physiology. Patients lacking either all peroxisomal functions or a single enzyme or …
Adulthood leukodystrophies
The leukodystrophies are a group of inherited white matter disorders with a heterogeneous
genetic background, considerable phenotypic variability and disease onset at all ages. This …
genetic background, considerable phenotypic variability and disease onset at all ages. This …
The changing face of adrenoleukodystrophy
J Zhu, F Eichler, A Biffi, CN Duncan… - Endocrine …, 2020 - academic.oup.com
Adrenoleukodystrophy (ALD) is a rare X-linked disorder of peroxisomal oxidation due to
mutations in ABCD1. It is a progressive condition with a variable clinical spectrum that …
mutations in ABCD1. It is a progressive condition with a variable clinical spectrum that …
The advanced lipoxidation end-product malondialdehyde-lysine in aging and longevity
The nonenzymatic adduction of malondialdehyde (MDA) to the protein amino groups leads
to the formation of malondialdehyde-lysine (MDALys). The degree of unsaturation of …
to the formation of malondialdehyde-lysine (MDALys). The degree of unsaturation of …
Enhanced axonal response of mitochondria to demyelination offers neuroprotection: implications for multiple sclerosis
Axonal loss is the key pathological substrate of neurological disability in demyelinating
disorders, including multiple sclerosis (MS). However, the consequences of demyelination …
disorders, including multiple sclerosis (MS). However, the consequences of demyelination …
[HTML][HTML] Peroxisomal ABC transporters: an update
ATP-binding cassette (ABC) transporters constitute one of the largest superfamilies of
conserved proteins from bacteria to mammals. In humans, three members of this family are …
conserved proteins from bacteria to mammals. In humans, three members of this family are …