Biomaterial Database

Stereological studies on the epiphyseal growth cartilage and characterization of costal cartilage proteoglycans in the achondroplastic (cn/cn) mouse. 1986

B Wikström, and A Hjerpe, and F P Reinholt, and B Engfeldt

The achondroplastic mouse is a dwarf mouse in which the endochondral growth of the skeleton is disturbed. The main morphological characteristic of the affected growth cartilage is an underdeveloped hypertrophic zone. The pattern of matrix mineralization seems to be unaffected, even in areas where hypertrophic chondrocytes are completely absent. The present stereological results indicate a disturbance of the cn/cn cartilage already in the proliferative zone. At the electron microscopical level a clearly abnormal spatial distribution of matrix vesicles was observed in the affected growth cartilage. The vesicle concentration of the cn/cn cartilage was increased, but the mineralizing cartilage showed a decrease of vesicles similar to that in the normal tissue. Parallel biochemical characterization of the proteoglycans and glycosaminoglycans of the cn/cn costal cartilage revealed normal conditions, also in the growth region, which was sampled as a separate tissue fraction. Thus the biochemical results provide no evidence of disturbed glycosaminoglycan metabolism.

UI	MeSH Term	Description	Entries
D008817	Mice, Mutant Strains	Mice bearing mutant genes which are phenotypically expressed in the animals.	Mouse, Mutant Strain,Mutant Mouse Strain,Mutant Strain of Mouse,Mutant Strains of Mice,Mice Mutant Strain,Mice Mutant Strains,Mouse Mutant Strain,Mouse Mutant Strains,Mouse Strain, Mutant,Mouse Strains, Mutant,Mutant Mouse Strains,Mutant Strain Mouse,Mutant Strains Mice,Strain Mouse, Mutant,Strain, Mutant Mouse,Strains Mice, Mutant,Strains, Mutant Mouse
D011509	Proteoglycans	Glycoproteins which have a very high polysaccharide content.	Proteoglycan,Proteoglycan Type H
D002358	Cartilage, Articular	A protective layer of firm, flexible cartilage over the articulating ends of bones. It provides a smooth surface for joint movement, protecting the ends of long bones from wear at points of contact.	Articular Cartilage,Articular Cartilages,Cartilages, Articular
D006132	Growth Plate	The area between the EPIPHYSIS and the DIAPHYSIS within which bone growth occurs.	Cartilage, Epiphyseal,Epiphyseal Cartilage,Epiphyseal Plate,Cartilages, Epiphyseal,Epiphyseal Cartilages,Epiphyseal Plates,Growth Plates,Plate, Epiphyseal,Plate, Growth,Plates, Epiphyseal,Plates, Growth
D006720	Homozygote	An individual in which both alleles at a given locus are identical.	Homozygotes
D006820	Hyaluronic Acid	A natural high-viscosity mucopolysaccharide with alternating beta (1-3) glucuronide and beta (1-4) glucosaminidic bonds. It is found in the UMBILICAL CORD, in VITREOUS BODY and in SYNOVIAL FLUID. A high urinary level is found in PROGERIA.	Amo Vitrax,Amvisc,Biolon,Etamucine,Healon,Hyaluronan,Hyaluronate Sodium,Hyvisc,Luronit,Sodium Hyaluronate,Acid, Hyaluronic,Hyaluronate, Sodium,Vitrax, Amo
D000130	Achondroplasia	An autosomal dominant disorder that is the most frequent form of short-limb dwarfism. Affected individuals exhibit short stature caused by rhizomelic shortening of the limbs, characteristic facies with frontal bossing and mid-face hypoplasia, exaggerated lumbar lordosis, limitation of elbow extension, GENU VARUM, and trident hand. (Online Mendelian Inheritance in Man, http://www.ncbi.nlm.nih.gov/Omim, MIM#100800, April 20, 2001)	Severe Achondroplasia with Developmental Delay and Acanthosis Nigricans,Achondroplasia, Severe, With Developmental Delay And Acanthosis Nigricans,SADDAN,SADDAN Dysplasia,Skeleton-Skin-Brain Syndrome,Achondroplasias,Dysplasia, SADDAN,Dysplasias, SADDAN,SADDAN Dysplasias,SADDANs,Skeleton Skin Brain Syndrome,Skeleton-Skin-Brain Syndromes,Syndrome, Skeleton-Skin-Brain,Syndromes, Skeleton-Skin-Brain
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
D012272	Ribs	A set of twelve curved bones which connect to the vertebral column posteriorly, and terminate anteriorly as costal cartilage. Together, they form a protective cage around the internal thoracic organs.	Rib
D014574	Uronic Acids	Acids derived from monosaccharides by the oxidation of the terminal (-CH2OH) group farthest removed from the carbonyl group to a (-COOH) group. (From Stedmans, 26th ed)	Uronic Acid,Acid, Uronic,Acids, Uronic