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BLOC1S4  -  biogenesis of lysosomal organelles complex...

Homo sapiens

Synonyms: BCAS4L, BLOC-1 subunit 4, BLOS4, Biogenesis of lysosome-related organelles complex 1 subunit 4, CNO, ...
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Disease relevance of CNO

  • The majority of the Down syndrome fetuses had CRL, FL and CNO values within the range of those for normal age-matched fetuses [1].
  • Results of analysis of variance (ANOVA) of central obesity status (CNO = no, CO = yes) and BMI (BMI tertiles used as a categorical variable) with these metabolic variables revealed that CO status had a significant effect (p < 0.05) on TC, VLDL-C, FPG and FTG [2].

Psychiatry related information on CNO


High impact information on CNO

  • We performed mutation screening of patients with HPS and failed to identify any CNO defects [4].
  • Levels of globin carbamylation of 0.5 moles CNO/mole hemoglobin, shown to be clinically effective in prolonging the life span of sickle erythrocytes, are obtained under these conditions and reach maximal levels after only 30 min of incubation [5].
  • Red blood cells exposed to cyanate (CNO) in vitro have a concentration-dependent decreased cell survival time associated with an inhibition of the ability of the cell membrane to synthesize lipids [5].
  • The values of K1K2 from kinetics are in good agreement with those obtained from spectral and [O2] determinations of equilibrated mixtures of Hr, CNO-, and O2 [6].
  • Impairment of this arm of neutrophil bactericidal activity by CNO- formed from urea may be one factor in the risk of urinary-tract infection associated with urinary stasis and perhaps in the generalized increase in susceptibility to infection in uraemic patients [7].

Biological context of CNO

  • In the former case it has been shown that "metal poisons" such as CN- and CNO- are not directly coordinated to the active site Zn(II) ion whereas spectroscopic studies indicate otherwise [8].
  • During the same exhalation, ENO(b/o) was compared to CNO at multiple sites in the airway as the catheter was progressively withdrawn [9].
  • These stages are related partly to general parameters of fetal development, such as M age (menstrual age), CRL (crown-rump length), CNO (composite number of ossified bones in hand and foot) and partly to stages in the skeletal development of the symphysis menti (SM I to SM V) [10].

Anatomical context of CNO

  • We have explored the possibility that CNO- might be one of the unknown substances responsible for the reported impairment, by urine and uraemic plasma, of neutrophil oxidative metabolism (especially as measured by luminol-enhanced chemiluminescence) [7].
  • Cyanate (CNO-) forms spontaneously in solutions containing urea, and is present in urine and the body fluids of uraemic patients [7].
  • As the catheter was withdrawn, mean CNO concentration progressively increased both in absolute values and as a proportion of ENO(b/o), until in the oropharynx, it was 96.1 +/- 5.2% ENO(b/o) [9].
  • A similar mechanism of cation is proposed for some other inhibitors of gastric acid secretion, e.g. nitrite (NO2-), cyanate (CNO-) and NH4+ [11].
  • The material available did not justify a description of skeletal development of the maxilla as a function of CRL or CNO [12].

Associations of CNO with chemical compounds

  • The larger univalent anions such as HCO3-, Br-, Cl-, NO3-, CNO- and I- seemed most effective, whereas the bivalent anion SO42- was relatively ineffective at 0.1 M, as were acetate and formate [13].
  • The heterotrophic and autotrophic alkaliphiles that grew with thiocyanate as an N source possessed a relatively high level of cyanase activity which converted cyanate (CNO-) to ammonia and CO2 [14].
  • Functional groups imaged by negative ion TOF-SIMS on (111) faces of the octahedral platelets were predominantly Cl and CN, with smaller amounts of C(2)H and CNO [15].
  • Our results show that the product P1 (CO2 + CNO) is the major product with absolute yield, while the product P4 (2CO + NO) is the minor product with less abundance [16].
  • Our data indicate that CNO quenches selectively one or two tryptophan residue(s) in the active site [17].

Analytical, diagnostic and therapeutic context of CNO

  • The crown-rump length (CRL), foot length (FL) and number of ossified bones in the hand and foot (CNO) were recorded [1].


  1. A histological and radiological investigation of the nasal bone in fetuses with Down syndrome. Tuxen, A., Keeling, J.W., Reintoft, I., Fischer Hansen, B., Nolting, D., Kjaer, I. Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology. (2003) [Pubmed]
  2. Central obesity and coronary risk factors. Ghosh, A., Bose, K., Chakravarti, S., Chaudhuri, A.B., Chattopadhyay, J., Dasgupta, G., Sengupta, S. Journal of the Royal Society of Health. (2004) [Pubmed]
  3. CNO and CEO decision-making patterns and compatability: Part 2, Using the MBTI. Freund, C.M. The Journal of nursing administration. (1989) [Pubmed]
  4. Cappuccino, a mouse model of Hermansky-Pudlak syndrome, encodes a novel protein that is part of the pallidin-muted complex (BLOC-1). Ciciotte, S.L., Gwynn, B., Moriyama, K., Huizing, M., Gahl, W.A., Bonifacino, J.S., Peters, L.L. Blood (2003) [Pubmed]
  5. Decreased life span and membrane damage of carbamylated erythrocytes in vitro. Lane, T.A., Burka, E.R. Blood (1976) [Pubmed]
  6. Conversion of oxy- into methemerythrin in the presence of anions. Bradić, Z., Conrad, R., Wilkins, R.G. J. Biol. Chem. (1977) [Pubmed]
  7. Cyanate-mediated inhibition of neutrophil myeloperoxidase activity. Qian, M., Eaton, J.W., Wolff, S.P. Biochem. J. (1997) [Pubmed]
  8. Is cyanate a carbonic anhydrase substrate? Supuran, C.T., Conroy, C.W., Maren, T.H. Proteins (1997) [Pubmed]
  9. A significant proportion of exhaled nitric oxide arises in large airways in normal subjects. Silkoff, P.E., McClean, P.A., Caramori, M., Slutsky, A.S., Zamel, N. Respiration physiology. (1998) [Pubmed]
  10. Development of deciduous mandibular incisors related to developmental stages in the mandible. Kjaer, I. Acta Odontol. Scand. (1980) [Pubmed]
  11. SCN-and HSCN transport through lipid bilayer membranes. A model for SCN- inhibition of gastric acid secretion. Gutknecht, J., Walter, A. Biochim. Biophys. Acta (1982) [Pubmed]
  12. Prenatal skeletal maturation of the human maxilla. Kjaer, I. J. Craniofac. Genet. Dev. Biol. (1989) [Pubmed]
  13. Inhibition of Bacillus cereus phospholipase C by univalent anions. Aakre, S.E., Little, C. Biochem. J. (1982) [Pubmed]
  14. Microbial thiocyanate utilization under highly alkaline conditions. Sorokin, D.Y., Tourova, T.P., Lysenko, A.M., Kuenen, J.G. Appl. Environ. Microbiol. (2001) [Pubmed]
  15. Morphology of gold nanoparticles synthesized by filamentous cyanobacteria from gold(I)-thiosulfate and gold(III)--chloride complexes. Lengke, M.F., Fleet, M.E., Southam, G. Langmuir : the ACS journal of surfaces and colloids. (2006) [Pubmed]
  16. Theoretical study on reaction mechanism of the ketenylidene radical with nitrogen dioxide. Zhang, J.X., Liu, J.Y., Li, Z.S., Sun, C.C. The journal of physical chemistry. A, Molecules, spectroscopy, kinetics, environment & general theory. (2005) [Pubmed]
  17. Interaction of tryptophan residues of cytochrome P450scc with a highly specific fluorescence quencher, a substrate analogue, compared to acrylamide and iodide. Lange, R., Anzenbacher, P., Müller, S., Maurin, L., Balny, C. Eur. J. Biochem. (1994) [Pubmed]
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