By Frederick W. Alt
Advances in Immunology, a fashioned and hugely revered book, provides present advancements in addition to entire experiences in immunology. Articles handle the wide variety of subject matters that contain immunology, together with molecular and mobile activation mechanisms, phylogeny and molecular evolution, and medical modalities. Edited and authored through the most important scientists within the box, every one quantity presents updated details and instructions for destiny * Contributions from top experts and specialists * Informs and updates on all of the most recent advancements within the box
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Additional info for Advances in Immunology, Vol. 105
The saposin function of GM2AP is required to stimulate the degradation of GM2 by b-hexosaminidase A (Hex-A) in vivo (Fig. 1) (Conzelmann and Sandhoff, 1979). Deficiency in GM2AP leads to the AB variant of GM2 gangliosidosis, an atypical form of Tay–Sachs disease with characteristic tissue accumulation of GM2 and related GSLs in neuronal lysosomes (Conzelmann and Sandhoff, 1978). , 1982). , 2000). , 2003). The most flexible of the loops contains the substrate-binding site and controls the entrance to the cavity to facilitate an open or a closed conformation.
1. Prosaposin pSAP is a 524-amino acid glycoprotein that contains a 16-residue signal peptide sequence and five glycosylation sites. , 1988). In humans, pSAP exists as an intracellular molecule of 68 kDa and a major extracellular form of 73 kDa. , 2003). , 1998). , 1992). This is not surprising considering their important functions in sphingolipid degradation. Protein expression analyses revealed high concentrations of pSAP in the adult liver and body fluids, especially in the brain, semen, milk, serum, pancreatic juice, and bile (Kolter and Sandhoff, 2005).
Ultimately, necrotic cells burst and release their organellar and cytosolic content into the surrounding tissue, which subsequently causes inflammation. , 2005). Accordingly, apoptotic cells shrink, condense their DNA and organelles prior to fragmentation, release membrane blebs, and finally disintegrate into apoptotic bodies. Thus, apoptosis avoids cell leakage and secondary harmful inflammation, and represents a ‘‘silent’’ way of death that cells undergo during development and tissue homeostasis (Ravichandran and Lorenz, 2007).