Proteins of Myelin

Lees, Marjorie B.; Brostoff, Steven W.

doi:10.1007/978-1-4757-1830-0_6

Cited by 243 publications

(184 citation statements)

References 155 publications

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“…In their monographic contributions to the field of myelin protein analysis, Lees and Brostoff (1984) as well as Norton and Cammer (1984) have discussed the problem concerning some discrepancies in the term of Wolfgram protein. The designation of Wolfgram fraction is generally used to describe a mixture of high mol wt components, whereas the term referred to as the Wolfgram protein is a doublet or triplet band observed on SDS gels at a position corresponding to a mol wt of 45-55 kDa.…”

Section: Discussionmentioning

confidence: 99%

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Myelin proteins in aging human brain

Wender¹,

Adamczewska-Goncerzewicz²,

Dorszewska³

1991

Molecular and Chemical Neuropathology

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Autopsy material of 13 persons who died between 70 and 89 yrs old and of patients who died between 27 and 44 yrs old was studied. White matter of temporal, parietal, and occipital lobes was investigated using histological and biochemical methods. According to results of neuropathological studies, the material of aged patients was divided into two subgroups: (a) brains with vascular changes only and (b) patients with senile atrophy of Alzheimer type. Chemical changes found in all studied brain lobes included a mild decrease in Wolfgram protein content with reciprocal increase in large basic protein content, together with a marked decrease in myelin yield. The abovementioned chemical changes were almost identical whether they were only vascular changes or whether senile atrophy of Alzheimer type was also present. It seems, therefore, that the degeneration of vessels is the decisive factor in the pathogenetic mechanism of myelin lesions in the aged brain.

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Section: Discussionmentioning

confidence: 99%

“…In this respect, Lees and Brostoff (1984) noted that the resolution of different gel systems differs significantly and, consequently, differences result in the studies conducted by various investigators. However, it is almost generally accepted that proteolipid and myelin basic protein contribute in 60 to 80% in total myelin proteins.…”

Section: Discussionmentioning

confidence: 99%

Myelin proteins in aging human brain

Wender¹,

Adamczewska-Goncerzewicz²,

Dorszewska³

1991

Molecular and Chemical Neuropathology

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“…These two regions were chosen on the basis of minimal codon redundancy upon reverse translation of the known amino acid sequence of bovine PLP (1). Accordingly, mixtures of 8 different 14-mers corresponding to residues 176-180 and 32 different 15-mers corresponding to residues 231-235 were end-labelled and used sequentially to screen the fetal bovine brain cDNA library.…”

Section: Isolation and Characterization Of Plp Cdna Clonesmentioning

confidence: 99%

“…Myelin of the central nervous system contains two major and several minor membrane proteins (1). A considerable amount of information has been accumulated in understanding the importance of these proteins in nervous function.…”

Section: Introductionmentioning

confidence: 99%

“…Studies have provided the complete primary structures of these two proteins (3)(4)(5)26), considerable insight into the arrangement of these proteins in association with the myelin membrane (1,6) and interesting suggestions as to their possible functions (7,8). However, little is known about the regulation of MBP and PLP synthesis and control of gene expression during myelination.…”

Section: Introductionmentioning

confidence: 99%

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Study of the expression of myelin proteolipid protein (lipophilin) using a cloned complementary DNA

et al. 1985

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We have prepared a XgtlO cDNA library with the mRNA isolated from fetal calf brains which were actively myelinating. Using two oligonucleotides made according to the known amino acid sequence of myelin proteolipid protein (PLP or lipophilin), we have isolated several cDNA clones for this major intrinsic membrane protein of myelin. One of these clones, designated as pLPl, is found to contain 444 bp of coding sequence for the C-terminal half of PLP and 486 bp of 3' untranslated sequence. Using pLPl as a hybridization probe, we have studied the regulation of PLP at the mRNA level during rat brain development. Our results show that the relative amounts of mRNA for PLP and that for the major extrinsic protein of the myelin membrane, myelin basic protein, increase coordinately during the course of myelination in the brain.

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Late-onset neurodegeneration in mice with increased dosage of the proteolipid protein gene

et al. 1998

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Mutations of the proteolipid protein (Plp) gene cause a generalized central nervous system (CNS) myelin deficit in Pelizaeus-Merzbacher disease of man and various tremor syndromes in animal models. X-linked spastic paraplegia is also due to Plp gene mutations but has a different clinical profile and more restricted pathology involving specific tracts and regions. We have shown previously that PLP overexpression in mice homozygous for a Plp transgene results in premature arrest of CNS myelination and premature death. Here, we demonstrate that a low-level increase in Plp gene expression in transgenic mice causes significant axonal degeneration and demyelination with predilection for specific tracts. Following normal motor development, aged mice develop progressive myelin loss, axonal swellings with resultant Wallerian degeneration, and marked vacuolation of the neuropil associated with ataxia, tremor, and seizures. The age of onset and severity of the phenotype is a function of Plp gene dosage. The corticospinal tracts, optic nerve, fasciculus gracilis cerebellum, and brainstem are particularly involved. Although oligodendrocyte cell bodies show little abnormality, their inner adaxonal tongue is often abnormal, suggesting a perturbation of the axon/glial interface that may underlie the axonal changes. We conclude that abnormal expression of an oligodendrocyte-specific gene can cause axonal damage, a finding that is relevant to the pathogenesis of PLP-associated disorders and probably to other myelin-related diseases.

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Proteins of Myelin

Cited by 243 publications

References 155 publications

Myelin proteins in aging human brain

Myelin proteins in aging human brain

Study of the expression of myelin proteolipid protein (lipophilin) using a cloned complementary DNA

Late-onset neurodegeneration in mice with increased dosage of the proteolipid protein gene

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