Biomaterial Database

Structure and function of peripheral nerve myelin proteins. 1995

K Uyemura, and H Asou, and Y Takeda

Department of Physiology, Keio University School of Medicine, Tokyo, Japan.

(1) Two glycoproteins, P0 and PASII, are widely distributed in the peripheral myelin, but not in the central myelin of mammals. P0-like protein is expressed in both peripheral and central myelins of some lower vertebrates, such as fish and tadpoles. A close relationship is suggested between P0 expression and neural regenerative activity. (2) PMP22 was reported to show high sequence homology, not only to PASII, but also to the growth arrest specific protein. Human PASII/PMP22 sequence was deduced and the locus of its gene, chromosome 17p12-p11.2, is similar to the region linked to Charcot-Marie-Tooth disease type 1A. (3) P0 expressed on cultured cells mediated strong homophilic cell adhesion and neurite outgrowth. Addition of the P0 glycopeptide inhibited cell adhesion markedly, indicating that the oligosaccharide with peptide is essential for P0 mediated cell adhesion. The active site for neurite outgrowth in P0 appears to be different from the adhesion site. (4) We determined the human chromosomal locus of the P0 gene, 1q22-q23, which corresponded to the locus of hereditary motor and sensory neuropathy, Charcot-Marie-Tooth disease type 1B. Point mutations in the extracellular domain of P0 are found in the patient's chromosome. (5) L1 is a large multifunctional adhesive glycoprotein of 200 kD. Rat and human L1 sequences confirmed a common structure for the mammalian nervous systems. An isoform of L1 (L1cs), lacking four amino acids, appears to localize in non-neuronal cells such as Schwann cells, while the complete L1 is exclusively found in neurons. L1cs in Schwann cells may be functionally different from L1 in neurons.

UI	MeSH Term	Description	Entries
D008969	Molecular Sequence Data	Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.	Sequence Data, Molecular,Molecular Sequencing Data,Data, Molecular Sequence,Data, Molecular Sequencing,Sequencing Data, Molecular
D009185	Myelin Proteins	MYELIN-specific proteins that play a structural or regulatory role in the genesis and maintenance of the lamellar MYELIN SHEATH structure.	Myelin Protein,Protein, Myelin,Proteins, Myelin
D010525	Peripheral Nerves	The nerves outside of the brain and spinal cord, including the autonomic, cranial, and spinal nerves. Peripheral nerves contain non-neuronal cells and connective tissue as well as axons. The connective tissue layers include, from the outside to the inside, the epineurium, the perineurium, and the endoneurium.	Endoneurium,Epineurium,Perineurium,Endoneuriums,Epineuriums,Nerve, Peripheral,Nerves, Peripheral,Perineuriums,Peripheral Nerve
D006023	Glycoproteins	Conjugated protein-carbohydrate compounds including MUCINS; mucoid, and AMYLOID glycoproteins.	C-Glycosylated Proteins,Glycosylated Protein,Glycosylated Proteins,N-Glycosylated Proteins,O-Glycosylated Proteins,Glycoprotein,Neoglycoproteins,Protein, Glycosylated,Proteins, C-Glycosylated,Proteins, Glycosylated,Proteins, N-Glycosylated,Proteins, O-Glycosylated
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000595	Amino Acid Sequence	The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.	Protein Structure, Primary,Amino Acid Sequences,Sequence, Amino Acid,Sequences, Amino Acid,Primary Protein Structure,Primary Protein Structures,Protein Structures, Primary,Structure, Primary Protein,Structures, Primary Protein
D000818	Animals	Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA.	Animal,Metazoa,Animalia
D012583	Schwann Cells	Neuroglial cells of the peripheral nervous system which form the insulating myelin sheaths of peripheral axons.	Schwann Cell,Cell, Schwann,Cells, Schwann
D013329	Structure-Activity Relationship	The relationship between the chemical structure of a compound and its biological or pharmacological activity. Compounds are often classed together because they have structural characteristics in common including shape, size, stereochemical arrangement, and distribution of functional groups.	Relationship, Structure-Activity,Relationships, Structure-Activity,Structure Activity Relationship,Structure-Activity Relationships