B6;129-Plp1^tm1Kan/J

Stock number: 003255
Product name: PLP KO
Research areas: Cancer Research, Cell Biology Research, Developmental Biology Research, Neurobiology Research

Description

These Plp1 knock-out mice exhibit no sign of motor deficits, tremors or seizures, in contrast to most naturally occurring PLP mutants.

Detailed description

The gene for myelin proteolipid protein (Plp) lies on the X chromosome. Mice homozygous or hemizygous for the Plp^tm1Kan targeted mutation are viable and fertile. Although they lack expression of the PLP protein, they show no sign of motor deficits, tremors or seizures, in contrast to most naturally occurring PLP mutants. PLP is a major membrane component of CNS myelin, and many PLP mutants show defects in myelin sheath formation. However, Plp^tm1Kan mice do not exhibit this dysmyelination. Some ultrastructural differences are observed in the myelin of Plp^tm1Kan mutant mice when compared to controls, including a less distinct difference between the major dense line (MDL) and the intraperiod line (IPL), which corresponds to reduced physical stability of the myelin sheath and suggests a function of PLP in maintenance of myelin architecture. Plp^tm1Kan mutant mice show no evidence of myelin breakdown with age.

Development

A 2.5 kb of intron 1 and the 3' end of exon 1 were removed, which also deleted the translational initiation codon (ATG) located adjacent to the first exon-intron boundary. The neomycin resistance gene is transcribed in antisense orientation relative to PLP. Targeting was performed in (129X1/SvJ x 129S1/Sv)F1-Kitl⁺-derived R1 ES cells. The strain originated on a mixed B6,129 background.

Allele

Symbol: Plp1^tm1Kan
Name: targeted mutation 1, Klaus-Armin Nave
MGI accession ID: MGI:1857446
Generation method: Targeted
Attributes: Null/Knockout
Synonyms: Plp-; PLP/DM20 null; PLP-null
Marker symbol: Plp1
Marker name: proteolipid protein (myelin) 1
Marker synonyms: DM20; GPM6C; HLD1; MMPL; PLP; PLP/DM20; PMD; SPG2
Chromosome: X
Strain of origin: (129X1/SvJ x 129S1/Sv)F1-Kitl⁺
Molecular note: A neomycin resistance cassette introduced by homologous recombination replaced the 3' part of exon 1,including the translation start codon and a 2.5 kb fragment of the first intron of Plp ("PLP/DM20 gene"). Western blot analysis showed absence of both PLP and DM20 proteolipids proteins in hemizygous mutant mice.