A lysosomal

COPD is a chronic inflammatory disease that results from prolonged and repeated inhalation of particles and gases, chronic (or latent) infection or an interaction of these factors. In many cases, the inflammation persists even when the exposure (in most cases smoking) is stopped. Prominent among the infiltrating leukocytes are neutrophils, CD8+ lymphocytes (Co-receptor for the T-cell receptor. CD8+ is specific for the class IMHC protein. It is expressed on the surface of cytotoxic T-cells and natural killer cells.) and CD68+ monocytic cells (A lysosomal antigen. All cells that rich in... 

Sialin was first identified as the product of the gene defective in sialidosis, a lysosomal storage disorder. The transporter mediates the movement of sialic acid out of lysosomes by coupling to the proton electrochemical gradient across the lysosomal membrane. Unlike the vesicular neurotransmitter transporters which are antiporters, sialin is a sympoiter with sialic acid and protons both moving out of the lysosome. 

E). Basally internalized conjugate (F) may also be directed to a lysosomal compartment (B) but, it may also cross the cell via a non-lysosomal proteolytic compartment (G) where the PLL moiety can be selectively cleaved. Free and intact HRP (H) can then be released into the apical medium. 

In some human studies where clinical chemistry measurements but no renal biopsies were performed, the only parameter of renal function shown to be affected was an increase in the levels of NAG in the urine. NAG is a lysosomal enzyme present in renal tubular cells that has been shown to be a sensitive indicator of early subclinical renal tubular disease. The mechanism by which lead affects the release of NAG from renal tubular cells is not known, but it is suggested that lead could attach to kidney cell membranes and alter membrane permeability (Chia et al. 1994). 

Aldurazyme (tradename, also known as laronidase) is a recombinant version of one polymorphic variant of the human enzyme a-L-iduronidase. It was approved for general medical use in the USA in 2003 and is indicated for the treatment of patients with certain forms of the rare inherited disease MPS I. MPS I is caused by a deficiency of a lysosomal a-L-iduronidase, which normally catalyses the hydrolysis of terminal a-L-iduronic acid residues from the glycosaminoglycans dermatan sulfate and heparin sulfate. The deficiency results in accumulation of the glycosaminoglycans throughout the body, causing widespread cell and tissue dysfunction. 

Familial demyelinative/dysmyelinative and axonal neuropathies may also be caused by impaired lysosomal lipid metabolism. Metachromatic leukodystrophy (sulfatide lipidosis) results from mutations of the arylsulfatase A gene, which encodes a lysosomal enzyme required for sulfatide turnover. Myelin is affected in both CNS and PNS, though dysfunction is restricted to the PNS in some patients, and the onset of symptoms can occur at any time between infancy and adulthood. Bone marrow transplantation can slow disease progression and improve nerve conduction velocities [57]. (See in Ch. 41.)... 

The neuronal ceroid lipofuscinoses (CLN), also referred to as Batten s disease, are a group of disorders characterized by the accumulation of autofluorescent lipopigments. Clinical hallmarks include blindness, seizures, cognitive and motor decline and early death. Age of onset varies from infancy to adulthood. Eight genetic forms have been identified [4]. Two involve lysosomal acid hydrolases. CLN1 codes for palmitoyl protein thioesterase 1. Clinically it presents most often in infancy and leads to loss of active movement and visual contact by 3 years of age. It is most common in Finland, where its incidence is 1 20,000. CLN2 codes for a lysosomal pepstatin-insensitive acid protease. 

Svendsen, C. and Weeks, J. M. (1995). The use of a lysosome assay for the rapid assessment of cellular stress from copper to the fresh water snail Viviparus contectus (Millet), Mar. Poll. Bull., 31, 139-142. 

Piguet, V., Gu, F., Foti, M., Demaurex, N., Gruenberg, J.-L. and Carpentier, J, and Trono, D. (1999). Nef-induced CD4 degradation a diacidic-based motif in Nef functions as a lysosomal targeting signal through the binding of /3-COP in endosomes. Cell 97, 63-73. 

Fig. 11.2 (a) HAADF-STEM image of a stained cell section (40nm thick). A SWNT cluster within a lysosome invading the lysosomal cell membrane, (b) Corresponding high-resolution lattice image of SWNTs at the lysosomal membrane from boxed area. Cytoplasm (cy) and secondary... 

EC 3.1.6.1) is a lysosomal enzyme that hydrolyzes sulfuric acid ester bonds. The enzyme exists in two forms, arylsulfatases A and B, that differ in substrate specificity and in sensitivity toward inhibitors [142][143]. Human tissues contain more arylsulfatase A than arylsulfatase B. The natural substrates of these enzymes are complex lipids such as cerebroside 3-sulfate, and gly-cosaminoglycans such as chondroitin 4-sulfate and derman sulfate [144], Deficiencies of these enzymes are associated with a number of lysosomal disorders. 

Phosphorylation of mannose residues important for directing an enzyme to a lysosome. 

Fusion of the endosome with a lysosome, accompanied by acidification and activation of lysosomal enzymes... 

Similarly, Chow et al. observed an increase in the lysozyme activity of a soluble lung fraction and of plasma after continuous exposure of rats to ozone at 0.8 ppm for 8 days. However, no difference in lung or plasma lysozyme activity from control values was present in rats continuously exposed to 0.2 or 0.5 ppm or intermittently exposed (0.2-0.8 ppm, 8 h/day for 7 days). Histochemical evidence of an increase in lung acid phosphatase, a lysosomal enzyme, has also been reported. ... 

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