ATP synthase activity in ovine ceroid lipofuscinosis [OCL6].

We measured ATP synthase activities in mitochondria isolated from livers of lambs with ceroid lipofuscinosis (OCL6) and compared them with those from similar isolations from obligate heterozygous and control lambs. Addition of excess Ca2+ to the incubation mixture resulted in an up-regulation of activity in mitochondria from control lambs but down-regulation in those from OCL6 affected lambs. The mean change in activity with Ca2+ for heterozygous animals was midway between those from control and affected groups being significantly different from control but not from affected.

Semiconducting biomaterials: current status and future perspectives.

The ability to truly interface living cells with intelligent semiconducting biomaterials will launch new post-digital technology involving biologically interfaced medical devices. This article examines the potential of porous silicon for use in cell-interfaced electronic devices.

Mutational spectra of tamoxifen-induced mutations in the livers of lacI transgenic rats.

Tamoxifen, an important drug in breast cancer treatment, causes liver cancer in rats. The standard range of in vitro tests have failed to show that it causes DNA damage, but 32P-postlabelling and DNA-binding studies have shown that tamoxifen forms DNA adducts in rat liver. In 1995 a transgenic rat (Big Blue; Stratagene, La Jolla, CA) became available which harbours the bacterial lacI gene, thereby allowing the in vivo study of tamoxifen mutagenesis.

Batten disease and the ATP synthase subunit c turnover pathway: raising antibodies to subunit c.

Analysis of storage bodies in the ceroid-lipofuscinoses (Batten disease) has demonstrated a high protein content suggestive of a proteinosis. Direct N-terminal sequencing has shown that subunit c of mitochondrial ATP synthase is specifically stored in the disease in sheep and cattle, and in the human late infantile and juvenile diseases, as well as in 3 breeds of dogs. No differences have been found between the stored subunit c and that in normal mitochondria.

Immunocytochemical studies in the ceroid-lipofuscinoses (Batten disease) using antibodies to subunit c of mitochondrial ATP synthase.

Immunocytochemistry, using antibodies against subunit c of mitochondrial ATP synthase, has been carried out in the ovine, canine, late infantile, and adult forms of ceroid-lipofuscinosis. Intensity of staining varied depending on the particular disease, species, fixation regime, and the antibody used. Differential staining of storage cytosomes in neurons of affected sheep and those in the late infantile patient suggested exposure of different epitopes.

Light transmission measurements and phototherapy eyepatches.

The transmission characteristics of phototherapy eyeshields have been measured under conditions that mimic the clinical situation. Peak transmission (less than 10%) was detected at 700 nm for the poorest patch tested and less than 2% for either of the other patches examined. The optics of measurement systems are considered with reference both to this and previous studies of light transmission through eyepatches.

Bovine ceroid-lipofuscinosis (Batten's disease): the major component stored is the DCCD-reactive proteolipid, subunit C, of mitochondrial ATP synthase.

The ceroid-lipofuscinoses (Batten's disease) are a group of recessively inherited lysosomal storage diseases of children and animals in which there is intracellular accumulation of a fluorescent lipopigment in a wide variety of cells. Lipopigment bodies isolated from pancreas, liver, kidney and brain tissue from a heifer affected with ceroid-lipofuscinosis contained between 55 and 62% protein. A dominant component comigrated on LDS-PAGE with the major low molecular weight protein stored in ovine ceroid-lipofuscinosis.

Transmission of light across the adult and neonatal eyelid in vivo.

Light transmission characteristics of the human adult and neonatal eyelid were measured in vivo. Light was delivered via a grating monochromator through a fibre-optic mounted onto a contact lens placed under the eyelid, and detected using a photodiode on its external skin surface. Data from 5 adult and 9 preterm neonatal subjects indicate that the eyelid acts as a predominantly red-pass filter, with mean transmissions at 700 nm of 14.

beta-Mannosidosis in a Salers calf: a new storage disease of cattle.

A storage disease characterised by widespread vacuolation of cells throughout the body was diagnosed in a newborn calf of the Salers breed. Extraction and analyses of water soluble material showed that the storage material was oligosaccharide in nature and was probably derived from the core region of the heterosaccharide moiety of glycoproteins. Glycosidase analyses showed that the disease was due to a deficiency of acidic beta-mannosidase.

Lysosomal storage of the DCCD reactive proteolipid subunit of mitochondrial ATP synthase in human and ovine ceroid lipofuscinoses.

The ceroid lipofuscinoses (Batten's disease) are a group of neuro-degenerative lysosomal storage diseases of children and animals that are recessively inherited. In the diseased individuals fluorescent storage bodies accumulate in a wide variety of cells, including neurons. The material stored in the cells of sheep affected with ceroid lipofuscinosis is two-thirds protein.

Light transmission through the human eyelid: in vivo measurement.

We describe an in vivo measurement technique for determining the transmission of light through the human eyelid and present data from three human subjects. The relevance of the transmission measurements are discussed in relation to (1) clinical electrophysiology and (2) the effects of light on the developing visual system.

Light transmission of phototherapy eyeshields.

Twelve types of phototherapy eyeshields showed peak light transmission of less than 0.1%, and none transmitted greater than 0.04% light in the 460 nm spectral region.

The effect of intrathecal iohexol on visual evoked response latency: a comparison including incidence of headache with iopamidol and metrizamide in myeloradiculography.

Fifty consecutive unselected patients referred for myeloradiculography and examined by the same radiologist, when facilities for measuring the visual evoked response were available, are considered. The effect on the visual evoked response of the examination and the incidence of headache following the use of iohexol as the contrast medium are compared with those after the use of iopamidol and metrizamide reported in a previous study. A total of 400 cases examined with iopamidol and 200 cases examined with iohexol are reviewed with regard to the incidence of headache.

Visual evoked response changes following intrathecal injection of water-soluble contrast media: a possible method of assessing neurotoxicity and a comparison of metrizamide and iopamidol.

An investigation was carried out to ascertain if there was a change in visual evoked responses following the intrathecal injection of water-soluble contrast media for myeloradiculography and if this change provided an indication of neurotoxicity as assessed by the onset of headache during a period of 20 h following the radiological examination. The patients were unselected and examined, when facilities for measuring the visual evoked response were available, immediately before and at 1 and 20 h after the examination. Control readings were carried out before, 1 h and 20 h after lumbar puncture in patients who did not have an injection of contrast medium.