A cellular mechanism governing the severity of Pelizaeus–Merzbacher disease

Degradation of CFTR by the ubiquitin-proteasome pathway

1995 • 1,285 citations

Protein Oligomerization in the Endoplasmic Reticulum

1989 • 993 citations

Posttranslational association of immunoglobulin heavy chain binding protein with nascent heavy chains in nonsecreting and secreting hybridomas.

1986 • 896 citations

Multiple proteolytic systems, including the proteasome, contribute to CFTR processing

1995 • 885 citations

The oligodendrocyte and its many cellular processes

1993 • 837 citations

Quality control in the secretory pathway

1995 • 679 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

Myelin: Biology and Chemistry

1992 • 436 citations

Regulation of Protein Export From the Endoplasmic Reticulum

1988 • 421 citations

X–linked spastic paraplegia and Pelizaeus–Merzbacher disease are allelic disorders at the proteolipid protein locus

1994 • 348 citations

Nucleotide sequences of two mRNAs for rat brain myelin proteolipid protein

1985 • 345 citations

A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency.

1994 • 293 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

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

1992 • 212 citations

Mutation of the proteolipid protein gene PLP in a human X chromosome-linked myelin disorder.

1989 • 205 citations

Degradation of proteins within the endoplasmic reticulum

1991 • 191 citations

Purification and characterization of human lysosomal membrane glycoproteins

1989 • 163 citations

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

1994 • 159 citations

Pelizaeus-Merzbacher disease: an X-linked neurologic disorder of myelin metabolism with a novel mutation in the gene encoding proteolipid protein.

1989 • 157 citations

Myelin‐specific proteolipid protein is expressed in myelinating schwann cells but is not incorporated into myelin sheaths

1987 • 154 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

Monoclonal Antibodies Against Myelin Proteolipid Protein: Identification and Characterization of Two Major Determinants

1991 • 142 citations

Genetics of Pelizaeus-Merzbacher Disease

1993 • 137 citations

Misfolded major histocompatibility complex class I molecules accumulate in an expanded ER-Golgi intermediate compartment.

1995 • 129 citations

Dysmyelination in transgenic mice resulting from expression of class I histocompatibility molecules in oligodendrocytes

1991 • 116 citations

Ultrastructure of the central nervous system in a myelin deficient rat

1982 • 110 citations

Pelizaeus-Merzbacher Disease: Clinical and Nosological Study

1986 • 105 citations

Arrest of proteolipid transport through the Golgi apparatus in Jimpy brain

1987 • 101 citations

Rumpshaker mouse: A new X-linked mutation affecting myelination: Evidence for a defect in PLP expression

1990 • 96 citations

Conservative amino acid substitution in the myelin proteolipid protein of jimpymsd mice.

1990 • 96 citations

Conservative amino acid substitution in the myelin proteolipid protein of jimpymsd mice

1990 • 85 citations

Intracellular transport and sorting of the oligodendrocyte transmembrane proteolipid protein

1994 • 77 citations

The initial events in myelin synthesis: orientation of proteolipid protein in the plasma membrane of cultured oligodendrocytes.

1989 • 76 citations

Hypomyelinated mutant mice: Description of jpmsd and comparison with jp and qk on their present genetic backgrounds

1980 • 70 citations

Pelizaeus-Merzbacher disease: a valine to phenylalanine point mutation in a putative extracellular loop of myelin proteolipid.

1991 • 66 citations

Proteolipid protein interactions in transfectants: Implications for myelin assembly

1994 • 62 citations

Genetic homogeneity of Pelizaeus-Merzbacher disease: tight linkage to the proteolipoprotein locus in 16 affected families. PMD Clinical Group.

1994 • 62 citations

Transport and assembly processes in the endoplasmic reticulum.

1990 • 61 citations

Immunocytochemical demonstration of the transport of myelin proteolipids through the Golgi apparatus

1983 • 60 citations

‘SHAKING PUPS’: A DISORDER OF CENTRAL MYELINATION IN THE SPANIEL DOG. III. QUANTITATIVE ASPECTS OF GLIA AND MYELIN IN THE SPINAL CORD AND OPTIC NERVE

1983 • 57 citations

EARLY LESION OF PELIZAEUS-MERZBACHER DISEASE

1973 • 53 citations

Pelizaeus-Merzbacher Disease: Classical or Connatal?

1991 • 52 citations

Transgenic mouse model for central nervous system demyelination.

1991 • 51 citations

Molecular diagnostics for myelin proteolipid protein gene mutations in Pelizaeus-Merzbacher disease.

1992 • 43 citations

A new mutation in the proteolipid protein (PLP) gene in a German family with pelizaeus‐merzbacher disease

1991 • 41 citations

Pelizaeus-Merzbacher disease: detection of mutations Thr181----Pro and Leu223----Pro in the proteolipid protein gene, and prenatal diagnosis.

1992 • 39 citations

Developmental expression of major myelin protein genes in the CNS of X‐Linked hypomyelinating mutant rumpshaker

1992 • 36 citations

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

1994 • 33 citations

The DM20 Protein of Myelin: Intracellular and Surface Expression Patterns in Transfectants

1992 • 31 citations

A Point Mutation at the X-Chromosomal Proteolipid Protein Locus in Pelizaeus-Merzbacher Disease Leads to Disruption of Myelinogenesis

1990 • 24 citations

Pelizaeus‐Merzbacher disease presenting as spinal muscular atrophy: Clinical and molecular studies

1994 • 23 citations

Paralytic tremor (pt) rabbit: a sex-linked mutation affecting proteolipid protein-gene expression

1993 • 22 citations

Proteolipid/DM-20 proteins bearing the paralytic tremor mutation in peripheral nerves and transfected Cos-7 cells

1996 • 18 citations