Osteoarthritis associated with mild chondrodysplasia in transgenic mice expressing alpha 1(IX) collagen chains with a central deletion

Type IX collagen, containing molecules of the three distinct polypeptides alpha 1(IX), alpha 2(IX), and alpha 3(IX), is an interesting hybrid extracellular matrix component in cartilage and eye tissues, with the properties of both a proteoglycan and a collagen. The alpha 1 (IX) chain has two forms, as a result of the tissue-specific utilization of two alternative promoters; the alpha 2(IX) chain carries a covalently attached glycosaminoglycan side chain. We have introduced a gene construct controlled by a tissue-specific promoter/enhancer and expressing a truncated alpha 1(IX) chain into mice. Examination of the offspring of two different founders revealed pathological changes similar to osteoarthritis in the articular cartilage of knee joints. In addition, mice homozygous for the transgene developed mild chondrodysplasia (i.e., mild dwarfism, anterior tonguing in the vertebral bodies, and ophthalmopathy). The relative ratio of transgene product to the endogenous alpha 1(IX) chain was approximately one in homozygotes and less than one in heterozygotes. Therefore, the phenotypic severity correlated well with the level of transgene expression. These findings suggest that mutations in type IX collagen genes may cause certain forms of osteoarthritis and chondrodysplasia in humans.     

Recalibration of the NBS Glass Standards of Spectral Transmittance

In 1934, Gibson, Walker, and Brown developed sets of four colored glass filters to serve as working standards of spectral transmittance for checking the reliability of spectrophotometers. Several sets of these glasses were measured carefully and reserved and designated as future reference standards. Duplicate standards evaluated by comparison with the reference standards are available by purchase to the public. The current set of reference standards was established in the years 1945 to 1947, and one of these reference standards (selenium-red) was recalibrated in 1952. This paper reports a recalibration, made in 1961 and 1962, of all four glasses (selenium-red, carbon-yellow, copper-green, cobalt-blue) on three spectrophotometers (Cary 14, Beckman DU, König-Martens). Except for the cobalt-blue standard, the values of spectral transmittance found differ from those previously assigned by amounts differing at some wavelengths by as much as or slightly more than the uncertainties estimated for the present values, though not by amounts exceeding the combined uncertainties of the present and previous determinations. The indicated changes for these three standards are fairly regular, however, and support the view that the selenium-red and carbon-yellow standards are changing chiefly by formation of a reflectance-reducing film on the surfaces. The indicated rate of upward drift is slow, and suggests that it takes about 10 years for the drift to exceed the assigned uncertainity.