Assembly of CNS Myelin in the Absence of Proteolipid Protein

Methods in Enzymology , Vol

1966 • 26,261 citations

Disruption of the proto-oncogene int-2 in mouse embryo-derived stem cells: a general strategy for targeting mutations to non-selectable genes

1988 • 1,690 citations

The oligodendrocyte and its many cellular processes

1993 • 837 citations

Quality control in the secretory pathway

1995 • 679 citations

Handbook of clinical neurology

1993 • 593 citations

Mouse P0 gene disruption leads to hypomyelination, abnormal expression of recognition molecules, and degeneration of myelin and axons

1992 • 493 citations

Immunoelectron microscopic localization of neural cell adhesion molecules (L1, N-CAM, and MAG) and their shared carbohydrate epitope and myelin basic protein in developing sciatic nerve.

1986 • 482 citations

Quality control in the secretory pathway: retention of a misfolded viral membrane glycoprotein involves cycling between the ER, intermediate compartment, and Golgi apparatus.

1994 • 447 citations

Expression of a myelin basic protein gene in transgenic shiverer mice: Correction of the dysmyelinating phenotype

1987 • 389 citations

Nucleotide sequences of two mRNAs for rat brain myelin proteolipid protein

1985 • 345 citations

Role of myelin Po protein as a homophilic adhesion molecule

1990 • 344 citations

Chromosomal mapping of mouse myelin basic protein gene and structure and transcription of the partially deleted gene in shiverer mutant mice

1985 • 331 citations

Splice site selection in the proteolipid protein (PLP) gene transcript and primary structure of the DM-20 protein of central nervous system myelin.

1987 • 300 citations

Oligodendrocytes originate in a restricted zone of the embryonic ventral neural tube defined by DM-20 mRNA expression

1995 • 296 citations

Proteins of Myelin

1977 • 281 citations

Premature arrest of myelin formation in transgenic mice with increased proteolipid protein gene dosage

1994 • 277 citations

Glial cell degeneration and hypomyelination caused by overexpression of myelin proteolipid protein gene

1994 • 263 citations

Proteolipid protein (PLP) of CNS myelin: positions of free, disulfide-bonded, and fatty acid thioester-linked cysteine residues and implications for the membrane topology of PLP

1992 • 227 citations

Disruption of the compacted myelin sheath of axons of the central nervous system in proteolipid protein-deficient mice.

1994 • 222 citations

Jimpy mutant mouse: a 74-base deletion in the mRNA for myelin proteolipid protein and evidence for a primary defect in RNA splicing.

1986 • 219 citations

Oligodendroglial cell death in jimpy mice: an explanation for the myelin deficit

1986 • 218 citations

Uncoupling of hypomyelination and glial cell death by a mutation in the proteolipid protein gene

1992 • 212 citations

Morphology of Myelin and Myelination

1984 • 189 citations

PELIZAEUS-MERZBACHER DISEASE

1964 • 183 citations

Aberrant splicing of proteolipid protein mRNA in the dysmyelinating jimpy mutant mouse.

1987 • 174 citations

Many naturally occurring mutations of myelin proteolipid protein impair its intracellular transport

1994 • 159 citations

Adhesive properties of proteolipid protein are responsible for the compaction of CNS myelin sheaths

1995 • 152 citations

Complete deletion of the proteolipid protein gene (PLP) in a family with X-linked Pelizaeus-Merzbacher disease.

1991 • 152 citations

A proteolipid protein gene family: Expression in sharks and rays and possible evolution from an ancestral gene encoding a pore-forming polypeptide

1993 • 150 citations

Mice doubly deficient in the genes for P0 and myelin basic protein show that both proteins contribute to the formation of the major dense line in peripheral nerve myelin

1995 • 150 citations

Monoclonal Antibody O10 Defines a Conformationally Sensitive Cell-Surface Epitope of Proteolipid Protein (PLP): Evidence that PLP Misfolding Underlies Dysmyelination in Mutant Mice

1996 • 145 citations

Molecular cloning of M6: Identification of a PLP/DM20 gene family

1993 • 143 citations

Abnormal compact myelin in the myelin-deficient rat: absence of proteolipid protein correlates with a defect in the intraperiod line.

1987 • 142 citations

Selective Expression of DM‐20, an Alternatively Spliced Myelin Proteolipid Protein Gene Product, in Developing Nervous System and in Nonglial Cells

1992 • 141 citations

Molecular Organization of Myelin

1984 • 141 citations

Genetics of Pelizaeus-Merzbacher Disease

1993 • 137 citations

A duplicated PLP gene causing Pelizaeus-Merzbacher disease detected by comparative multiplex PCR.

1996 • 118 citations

Cell dispersion patterns in different cortical regions studied with an X-inactivated transgenic marker

1995 • 117 citations

Monoclonal M6 antibody interferes with neurite extension of cultured neurons

1992 • 112 citations

[46] Isolation of myelin from nerve tissue

1974 • 109 citations

Parallel Evolution and Coexpression of the Proteolipid Proteins and Protein Zero in Vertebrate Myelin

1996 • 105 citations

Diffraction Studies of Molecular Organization and Membrane Interactions in Myelin

1984 • 104 citations

Arrest of proteolipid transport through the Golgi apparatus in Jimpy brain

1987 • 101 citations

Myelination in the jimpy mouse in the absence of proteolipid protein

1989 • 90 citations

Shaking pups: a disorder of central myelination in the spaniel dog. II. Ultrastructural observations on the white matter of the cervical spinal cord

1981 • 86 citations

Inherited Primary Peripheral Neuropathies

1993 • 83 citations

X-Linked Developmental Defects of Myelination: From Mouse Mutants to Human Genetic Diseases

1996 • 83 citations

Dominant-negative action of the jimpy mutation in mice complemented with an autosomal transgene for myelin proteolipid protein.

1995 • 78 citations

A Combination of PLP and DM20 Transgenes Promotes Partial Myelination in the Jimpy Mouse

1994 • 67 citations

Myelin proteolipid protein gene structure and its regulation of expression in normal and jimpy mutant mice

1988 • 67 citations

Myelin structure in proteolipid protein (PLP)-null mouse spinal cord

1996 • 64 citations

Decompaction of CNS myelin leads to a reduction of the conduction velocity of action potentials in optic nerve

1995 • 61 citations

Programmed Cell Death in the Dysmyelinating Mutants

1995 • 58 citations

Oligodendrocyte progenitors in the embryonic spinal cord express DM‐20

1996 • 50 citations

Molecular Basis of Common Hereditary Motor and Sensory Neuropathies in Humans and in Mouse Models

1995 • 50 citations

EMA: A developmentally regulated cell‐surface glycoprotein of CNS neurons that is concentrated at the leading edge of growth cones

1992 • 49 citations

Jimpy Mice: Quantitation of Myelin‐Associated Glycoprotein and Other Proteins

1986 • 48 citations

Methods in Enzymology, Volume 61

1980 • 41 citations

Rapid preimplantation detection of mutant (shiverer) and normal alleles of the mouse myelin basic protein gene allowing selective implantation and birth of live young.

1990 • 40 citations

Overexpression of DM20 messenger RNA in two brothers with pelizaeus‐merzbacher disease

1995 • 37 citations

The genetics of myelin

1993 • 35 citations

Paralytic Tremor (pt): A New Allele of the Proteolipid Protein Gene in Rabbits

1994 • 33 citations

Analysis of myelin proteolipid protein and F0ATPase subunit 9 in normal andjimpy CNS

1994 • 15 citations

Basic biology of the Schwann cell

1995 • 14 citations

Isolation and identification of proteolipid proteins injimpy mouse brain

1994 • 13 citations