Premium
Immunohistochemical Investigations on Cerebellar Purkinje Cells of Menkes' Kinky Hair Disease: Disappearance of Inositol 1, 4, 5‐Triphosphate Receptor Protein, and Expression of Phosphorylated Neurofilament Proteins, αB‐Crystallin and Stress‐response Proteins
Author(s) -
Kato Shinsuke,
Ito Masayuki,
Ohama Eisaku,
Mikoshiba Katsuhiko,
Maeda Nobuaki,
Hirano Asao
Publication year - 1993
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/j.1440-1789.1993.tb00213.x
Subject(s) - neurofilament , purkinje cell , immunohistochemistry , cerebellum , biology , phosphorylation , inositol , antibody , receptor , microbiology and biotechnology , biochemistry , endocrinology , immunology
Immunohistochemical investigations were carried out on cerebellar Purkinje cells in patients with Menkes' kinky hair disease (MD) and normal control subjects (ages: 1–65 years). Six antibodies were used. They were specific to inositol 1,4,5‐triphosphate receptor protein regarded as P 400 (P 400 /IP 3 R), phosphorylated neurofilament proteins (pNFP), αB‐crystallin, stress‐response protein (srp) 72, srp 27 and ubiquitin, respectively. In normal brains, all Purkinje cell bodies, axons, and dendrites including the spiny branchlets of dendrites were positive for anti‐P 400 /IP 3 R antibody. In comparison, not every Purkinje cells in MD cases had P 400 /IP 3 R immunoreactivity. And some of the cells were more intensely stained than others. The immunoreaction products of P 400 /IP 3 R were frequently detected in cell bodies and proximal portions of the dendrites, but not in the peripheral dendrites including spiny branchlets. Most of the Purkinje cell bodies in MD reacted with anti‐pNFP antibody, while only a small fraction of the cells was positive with anti‐αB‐crystallin and srp 72 and srp 27. Ubiquitin immunoreactivity was not detected. In contrast, Purkinje cell bodies from normal subjects were not recognized by antibody to pNFP, αB‐crystallin, srp 72 and srp 27. Our results indicate that MD is associated with the loss of inositol 1,4,5‐triphosphate receptor in Purkinje cells, and an aberrant phosphorylation of neurofilament proteins or aberrant distribution of normal phosphorylated neurofilament proteins. These impairments may lead to the expression of stress response proteins or αB‐crystallin in some cells.