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Gene Review

LYST  -  lysosomal trafficking regulator

Homo sapiens

Synonyms: Beige homolog, CHS, CHS1, Lysosomal-trafficking regulator
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Disease relevance of LYST

  • Two novel CHS1 (LYST) mutations: clinical correlations in an infant with Chediak-Higashi syndrome [1].
  • The two enzymes expressed as single species in Escherichia coli synthesized both products, and both were also obtained with a CHS heterodimer containing a single active site [2].
  • BACKGROUND: The purpose of this study was to characterize the relationship between tissue cytokine expression and the cellular infiltrate present in chronic hyperplastic sinusitis with nasal polyposis (CHS/NP) and to compare the immunopathology and cytokine profile of patients with allergy versus patients without allergy [3].
  • Use of sonography to evaluate carotid atherosclerosis in the elderly. The Cardiovascular Health Study. CHS Collaborative Research Group [4].
  • Adult identified with congenital central hypoventilation syndrome--mutation in PHOX2b gene and late-onset CHS [5].

Psychiatry related information on LYST

  • We examined mortality in 3602 participants of the Cardiovascular Health (CHS) Cognition Study in four US communities evaluated for dementia incidence between 1992 and 1999 and followed for 6.5 years [6].
  • Initially, the CYT cohort scores indicated greater severity on several substance-related measures, while the CHS cohort scored higher on prior mental health treatment, victimization, and illegal activities measures [7].

High impact information on LYST

  • Our results show that both Lyst and Lys- Lp(a)s and their derived apo(a)s, bound to PM-fibrinogen with similar affinities (Kds: 33-100 nM), whereas the B(max) values were threefold higher for apo(a)s. Both the lysine analog epsilon-aminocaproic acid and L-proline inhibited the binding of Lp(a) and apo(a) to PM fibrinogen [8].
  • The Chediak-Higashi syndrome (CHS) is a human recessive autosomal disease caused by mutations in a single gene encoding a protein of unknown function, called lysosomal-trafficking regulator [9].
  • These results suggest that the product of the Lyst gene is required for sorting endosomal resident proteins into late multivesicular endosomes by a mechanism involving microtubules [9].
  • Plant-specific polyketide synthase genes constitute a gene superfamily, including universal chalcone synthase [CHS; malonyl-CoA:4-coumaroyl-CoA malonyltransferase (cyclizing) (EC] genes, sporadically distributed stilbene synthase (SS) genes, and atypical, as-yet-uncharacterized CHS-like genes [10].
  • Enzyme activity analysis of proteins produced in vitro indicates that the GCHS2 reaction is a non-SS variant of the CHS reaction, with both different substrate specificity (to benzoyl-CoA) and a truncated catalytic profile [10].

Chemical compound and disease context of LYST

  • Zonal inhibition of E coli growth was proportional to the concentration of ciprofloxacin on the CHS [11].
  • Current estrogen-progestin and estrogen replacement therapy in elderly women: association with carotid atherosclerosis. CHS Collaborative Research Group. Cardiovascular Health Study [12].
  • In the context of the Citizen Health System (CHS) project, a modular Medical Contact Center (MCC) was developed, which can be used in the monitoring, treatment, and management of chronically ill patients at home, such as diabetic or congestive heart failure patients [13].

Biological context of LYST

  • MATERIALS AND METHODS: Fourteen cDNA fragments, covering the entire coding domain of LYST, were used as baits to screen five human cDNA libraries by a yeast two-hybrid method, modified to allow screening in the activation and the binding domain, three selectable markers, and more stringent confirmation procedures [14].
  • The goal of this study was to identify proteins that interact with LYST as a first step in understanding how LYST modulates lysosomal exocytosis [14].
  • Chediak-Higashi syndrome is an autosomal recessive, immune deficiency disorder of human (CHS) and mouse (beige, bg) that is characterized by abnormal intracellular protein transport to, and from, the lysosome [15].
  • Novel mutations were identified within the region of the coding domain common to both isoforms in three CHS patients: C-->T transitions that generated stop codons (R50X and Q1029X) were found in two patients, and a novel frameshift mutation (deletion of nucleotides 3073 and 3074 of the coding domain) was found in a third [15].
  • Here, we describe the organization and genomic DNA sequence of the CHS1 gene and mutation analysis of 21 unrelated patients with the childhood, adolescent, and adult forms of CHS [16].

Anatomical context of LYST

  • CONCLUSIONS:On the basis of protein interactions, LYST appears to function as an adapter protein that may juxtapose proteins that mediate intracellular membrane fusion reactions [14].
  • Neurobeachin's subcellular localization, its coat protein-like membrane recruitment, and its sequence similarity to LYST suggest an involvement in neuronal post-Golgi membrane traffic, one of its functions being to recruit protein kinase A to the membranes with which it associates [17].
  • Further insight into a possible mechanism of this transport defect came from immunolocalizing the lysosomal trafficking regulator protein, as antibodies directed to a peptide from its COOH terminal domain decorated punctated structures partially aligned along microtubules [9].
  • BACKGROUND:Chediak-Higashi syndrome (CHS) is an inherited immunodeficiency disease characterized by giant lysosomes and impaired leukocyte degranulation [14].
  • One family of genes, including LYST and HPS/ep, shows the relationship between melanosomes and lysosomes [18].

Associations of LYST with chemical compounds

  • Associations of postmenopausal estrogen use with cardiovascular disease and its risk factors in older women. The CHS Collaborative Research Group [19].
  • In co-action with a monomeric reductase, CHS also synthesizes 6'-deoxychalcone, but with the chalcone as second product when using plant preparations [2].
  • Inward turbulent particle transport observed in the rf heated plasma of the H-1 toroidal heliac is reproduced in the CHS heliotron/torsatron by generating a region of positive radial electric field shear (E'(r)>0) using electron cyclotron resonance heating of the plasma edge [20].
  • Epidemiological studies indicate that the use of nonnutritive sweeteners (CHS and saccharin) has not resulted in a measurable overall increase in the risk of bladder cancer in individuals who have ever used these products [21].
  • In contrast, recombinant chalcone synthase (CHS; EC from the same cell cultures preferentially used 4-coumaroyl-CoA and also converted CoA esters of benzoic acids [22].

Analytical, diagnostic and therapeutic context of LYST

  • Northern blots of lymphoblastoid mRNA from CHS patients revealed loss of the largest transcript (approximately 13.5 kb) in two of seven CHS patients, while the small mRNA was undiminished in abundance [15].
  • Western blot analysis suggests that the Lyst(bg-grey) mutation causes instability of the LYST protein [23].
  • The Chediak-Higashi syndrome (CHS), a life-threatening autosomal recessive disease with frequent mutations in the LYST gene, and its animal model, the beige mouse, are both characterized by lysosomal defects with accumulation of giant lysosomes [24].
  • In order to identify the mutation within the LYST gene causing CHS in cattle, cDNA fragments of the LYST gene were amplified from an affected animal by RT-PCR and their nucleotide sequences were completely determined [25].
  • The ultimate diagnostic test is to look for a mutated LYST gene [26].


  1. Two novel CHS1 (LYST) mutations: clinical correlations in an infant with Chediak-Higashi syndrome. Zarzour, W., Kleta, R., Frangoul, H., Suwannarat, P., Jeong, A., Kim, S.Y., Wayne, A.S., Gunay-Aygun, M., White, J., Filipovich, A.H., Gahl, W.A. Mol. Genet. Metab. (2005) [Pubmed]
  2. Reaction mechanisms of homodimeric plant polyketide synthase (stilbenes and chalcone synthase). A single active site for the condensing reaction is sufficient for synthesis of stilbenes, chalcones, and 6'-deoxychalcones. Tropf, S., Kärcher, B., Schröder, G., Schröder, J. J. Biol. Chem. (1995) [Pubmed]
  3. Evidence for distinct cytokine expression in allergic versus nonallergic chronic sinusitis. Hamilos, D.L., Leung, D.Y., Wood, R., Cunningham, L., Bean, D.K., Yasruel, Z., Schotman, E., Hamid, Q. J. Allergy Clin. Immunol. (1995) [Pubmed]
  4. Use of sonography to evaluate carotid atherosclerosis in the elderly. The Cardiovascular Health Study. CHS Collaborative Research Group. O'Leary, D.H., Polak, J.F., Wolfson, S.K., Bond, M.G., Bommer, W., Sheth, S., Psaty, B.M., Sharrett, A.R., Manolio, T.A. Stroke (1991) [Pubmed]
  5. Adult identified with congenital central hypoventilation syndrome--mutation in PHOX2b gene and late-onset CHS. Weese-Mayer, D.E., Berry-Kravis, E.M., Zhou, L. Am. J. Respir. Crit. Care Med. (2005) [Pubmed]
  6. Survival following dementia onset: Alzheimer's disease and vascular dementia. Fitzpatrick, A.L., Kuller, L.H., Lopez, O.L., Kawas, C.H., Jagust, W. J. Neurol. Sci. (2005) [Pubmed]
  7. Comparing outcomes of best-practice and research-based outpatient treatment protocols for adolescents. Godley, S.H., Jones, N., Funk, R., Ives, M., Passetti, L.L. Journal of psychoactive drugs. (2004) [Pubmed]
  8. Evidence that the fibrinogen binding domain of Apo(a) is outside the lysine binding site of kringle IV-10: a study involving naturally occurring lysine binding defective lipoprotein(a) phenotypes. Klezovitch, O., Edelstein, C., Scanu, A.M. J. Clin. Invest. (1996) [Pubmed]
  9. Deficient peptide loading and MHC class II endosomal sorting in a human genetic immunodeficiency disease: the Chediak-Higashi syndrome. Faigle, W., Raposo, G., Tenza, D., Pinet, V., Vogt, A.B., Kropshofer, H., Fischer, A., de Saint-Basile, G., Amigorena, S. J. Cell Biol. (1998) [Pubmed]
  10. Duplication and functional divergence in the chalcone synthase gene family of Asteraceae: evolution with substrate change and catalytic simplification. Helariutta, Y., Kotilainen, M., Elomaa, P., Kalkkinen, N., Bremer, K., Teeri, T.H., Albert, V.A. Proc. Natl. Acad. Sci. U.S.A. (1996) [Pubmed]
  11. Effect of antibiotic-loaded hydrophilic stent in the prevention of bacterial adherence: a study of the charge, discharge, and recharge concept using ciprofloxacin. Leung, J.W., Liu , Y., Cheung , S., Chan, R.C., Inciardi, J.F., Cheng, A.F. Gastrointest. Endosc. (2001) [Pubmed]
  12. Current estrogen-progestin and estrogen replacement therapy in elderly women: association with carotid atherosclerosis. CHS Collaborative Research Group. Cardiovascular Health Study. Jonas, H.A., Kronmal, R.A., Psaty, B.M., Manolio, T.A., Meilahn, E.N., Tell, G.S., Tracy, R.P., Robbins, J.A., Anton-Culver, H. Annals of epidemiology. (1996) [Pubmed]
  13. The Citizen Health System (CHS): a modular medical contact center providing quality telemedicine services. Maglaveras, N., Chouvarda, I., Koutkias, V.G., Gogou, G., Lekka, I., Goulis, D., Avramidis, A., Karvounis, C., Louridas, G., Balas, E.A. IEEE transactions on information technology in biomedicine : a publication of the IEEE Engineering in Medicine and Biology Society. (2005) [Pubmed]
  14. The Chediak-Higashi protein interacts with SNARE complex and signal transduction proteins. Tchernev, V.T., Mansfield, T.A., Giot, L., Kumar, A.M., Nandabalan, K., Li, Y., Mishra, V.S., Detter, J.C., Rothberg, J.M., Wallace, M.R., Southwick, F.S., Kingsmore, S.F. Mol. Med. (2002) [Pubmed]
  15. Identification of mutations in two major mRNA isoforms of the Chediak-Higashi syndrome gene in human and mouse. Barbosa, M.D., Barrat, F.J., Tchernev, V.T., Nguyen, Q.A., Mishra, V.S., Colman, S.D., Pastural, E., Dufourcq-Lagelouse, R., Fischer, A., Holcombe, R.F., Wallace, M.R., Brandt, S.J., de Saint Basile, G., Kingsmore, S.F. Hum. Mol. Genet. (1997) [Pubmed]
  16. Apparent genotype-phenotype correlation in childhood, adolescent, and adult Chediak-Higashi syndrome. Karim, M.A., Suzuki, K., Fukai, K., Oh, J., Nagle, D.L., Moore, K.J., Barbosa, E., Falik-Borenstein, T., Filipovich, A., Ishida, Y., Kivrikko, S., Klein, C., Kreuz, F., Levin, A., Miyajima, H., Regueiro, J., Russo, C., Uyama, E., Vierimaa, O., Spritz, R.A. Am. J. Med. Genet. (2002) [Pubmed]
  17. Neurobeachin: A protein kinase A-anchoring, beige/Chediak-higashi protein homolog implicated in neuronal membrane traffic. Wang, X., Herberg, F.W., Laue, M.M., Wullner, C., Hu, B., Petrasch-Parwez, E., Kilimann, M.W. J. Neurosci. (2000) [Pubmed]
  18. Homologous pigmentation mutations in human, mouse and other model organisms. Jackson, I.J. Hum. Mol. Genet. (1997) [Pubmed]
  19. Associations of postmenopausal estrogen use with cardiovascular disease and its risk factors in older women. The CHS Collaborative Research Group. Manolio, T.A., Furberg, C.D., Shemanski, L., Psaty, B.M., O'Leary, D.H., Tracy, R.P., Bush, T.L. Circulation (1993) [Pubmed]
  20. Inward turbulent transport produced by positively sheared radial electric field in stellarators. Shats, M.G., Toi, K., Ohkuni, K., Yoshimura, Y., Osakabe, M., Matsunaga, G., Isobe, M., Nishimura, S., Okamura, S., Matsuoka, K. Phys. Rev. Lett. (2000) [Pubmed]
  21. Assessment of the carcinogenicity of the nonnutritive sweetener cyclamate. Ahmed, F.E., Thomas, D.B. Crit. Rev. Toxicol. (1992) [Pubmed]
  22. Benzophenone synthase and chalcone synthase from Hypericum androsaemum cell cultures: cDNA cloning, functional expression, and site-directed mutagenesis of two polyketide synthases. Liu, B., Falkenstein-Paul, H., Schmidt, W., Beerhues, L. Plant J. (2003) [Pubmed]
  23. Grey, a novel mutation in the murine Lyst gene, causes the beige phenotype by skipping of exon 25. Runkel, F., Büssow, H., Seburn, K.L., Cox, G.A., Ward, D.M., Kaplan, J., Franz, T. Mamm. Genome (2006) [Pubmed]
  24. A missense mutation in the WD40 domain of murine Lyst is linked to severe progressive Purkinje cell degeneration. Rudelius, M., Osanger, A., Kohlmann, S., Augustin, M., Piontek, G., Heinzmann, U., Jennen, G., Russ, A., Matiasek, K., Stumm, G., Schlegel, J. Acta Neuropathol. (2006) [Pubmed]
  25. Cloning of bovine LYST gene and identification of a missense mutation associated with Chediak-Higashi syndrome of cattle. Kunieda, T., Nakagiri, M., Takami, M., Ide, H., Ogawa, H. Mamm. Genome (1999) [Pubmed]
  26. Chediak-Higashi syndrome: four cases from Northern Finland. Möttönen, M., Lanning, M., Baumann, P., Saarinen-Pihkala, U.M. Acta Paediatr. (2003) [Pubmed]
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