I-Cell

Morin, J. G., and Hastings, J. W. (1971a). Biochemistry of the bioluminescence of colonial hydroids and other coelenterates. I. Cell. Physiol. 77 305-311. 

Man 6-P receptors, located in the Golgi apparatus, bind the Man 6-P residue of these enzymes and direct them to the lysosomes. Fibroblasts from patients with I-cell disease (see below) are severely deficient in the activity of the GIcNAc phosphotransferase. 

I-Cell Disease Results From Faulty Targeting of Lysosomal Enzymes... 

As indicated above, Man 6-P serves as a chemical marker to target certain lysosomal enzymes to that organelle. Analysis of cultured fibroblasts derived from patients with I-cell (inclusion cell) disease played a large part in revealing the above role of Man 6-P. I-cell disease is an uncommon condition characterized by severe progressive psychomotor retardation and a variety of physical signs, with death often occurring in the first decade. Cultured cells from patients with I-cell disease were found to lack almost all of the normal lysosomal enzymes the lysosomes thus accumulate many different... 

Pseudo-Hurler polydystrophy is another genetic disease closely related to I-ceU disease. It is a milder condition, and patients may survive to adulthood. Smdies have revealed that the GlcNAc phosphotransferase involved in I-cell disease has several domains, including a catalytic domain and a domain that specifi-... 

Scriver CR et al (editors) The Metabolic and Molecular Bases of In-heritedDisease, 8th ed. McGraw-Hill, 2001.(Various chapters in this text give in-depth coverage of topics such as I-cell disease and disorders of glycoprotein degradation.)... 

The term mucolipidosis was introduced to denote diseases that combined features common to both mucopolysaccharidoses and sphingolipidoses (Chapter 24). Three mucolipidoses are listed in Table 48—7. In sialidosis (mucolipidosis I, ML-I), various oligosaccharides derived from glycoproteins and certain ganglio-sides can accumulate in tissues. I-cell disease (ML-II)... 

Table I. Cells in Which Arachidonic Acid Metabolism Is Stimulated by Palytoxin to Produce Cyclooxygenase Products ...

Methylmalonic aciduria (Bi2-reslstant) Mucolipidosis II (I-cell disease) Nlemann-Plck disease Sandhoff disease... 

In parental cells, pelDv.uidA expression in IP decreased when previously supplemented with PeCl3. In cbsE-I cells, expression of pelDv.uidA was higher than in parental cells and addition of PeCl3 into IP was ineffective. 

Electrolyte-concentration cells are based on electrolyte dilution, and have two identical electrodes that are immersed in two solutions of the same electrolyte containing ions of the electrode material at two different activities. Electrolyte concentration cells are classified as (i) cells without liquid junctions and (ii) cells with liquid junctions. 

Williams MC. Alveolar type I cells molecular phenotype and development. Annu Rev Physiol 2003 65 669-695. 

Fig. 20. Stage I cell schematic for simultaneous C02 scrubbing and 02 recovery.

Figure 1 Schematic diagrams illustrating the patch-clamp technique. (A) Overall setup for isolating single ionic channels in an intact patch of cell membrane. P = patch pipet R = reference microelectrode I = intracellular microelectrode Vp = applied patch potential Em = membrane potential Vm = Em — Vp = potential across the patch A = patch-clamp amplifier. (From Ref. 90.) (B) Five different recording configurations, and procedures used to establish them, (i) Cell attached or intact patch (ii) open cell attached patch (iii) whole cell recording (iv) excised outside-out patch (v) excised inside-out patch. Key i = inside of the cell o = outside of the cell. (Adapted from Ref. 283.)...

Terada, S., Tsujimoto, T., Takei, Y., Takahashi, T. and Hirokawa, N. Impairment of inhibitory synaptic transmission in mice lacking synapsin I. /. Cell Biol. 145 1039-1048, 1999. 

Mucolipidosis type 11 (I-cell disease) Mucolipidosis type IIIA (pseudo-Hurler s)... 

Hickman, S. and Neufeld, E. F. A hypothesis for I-cell disease defective hydrolases that do not enter lysosomes. Biochem. Biophys. Res. Commun. 49 992-999,1972. 

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