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Gene Review:

PDSS2 - prenyl (decaprenyl) diphosphate synthase,...

Homo sapiens

Synonyms: All-trans-decaprenyl-diphosphate synthase subunit 2, C6orf210, COQ10D3, Candidate tumor suppressor protein, DLP1, ...

Glaunsinger, B.A. et al., L??pez, L.C. et al., Senchenko, V. et al., Cooney, K.A. et al., Rai, S.K. et al., et al.

Armes, Hammet, de Silva, Ciciulla, Ramus, Soo, Mahoney, Yarovaya, Henderson, Gish, Hutchins, Price, Venter, Kwong, Lee, Wong, Zhou, Wong, Lo, Welch, Berkowitz, Mok, Mourtada-Maarabouni, Keen, Clark, Cooper, Williams,

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Disease relevance of PDSS2

Here, we describe an infant with severe Leigh syndrome, nephrotic syndrome, and CoQ(10) deficiency in muscle and fibroblasts and compound heterozygous mutations in the PDSS2 gene, which encodes a subunit of decaprenyl diphosphate synthase, the first enzyme of the CoQ(10) biosynthetic pathway [1].
Link of the unique oncogenic properties of adenovirus type 9 E4-ORF1 to a select interaction with the candidate tumor suppressor protein ZO-2 [2].
Candidate tumor suppressor HYAL2 is a glycosylphosphatidylinositol (GPI)-anchored cell-surface receptor for jaagsiekte sheep retrovirus, the envelope protein of which mediates oncogenic transformation [3].
Candidate tumor suppressor genes on 13q that may be important in the development of prostate cancer include the retinoblastoma susceptibility gene (RBI) and a gene associated with inherited breast cancer (BRCA2) [4].
The smallest region homozygously deleted in 3p21.3C was located between D3S1568 (CACNA2D2 gene) and D3S4604 (SEMA3F gene) and contains 17 genes previously defined as lung cancer candidate Tumor suppressor genes (TSG(s)) [5].

High impact information on PDSS2

The candidate tumor suppressor protein inhibitor of growth family member 4 (ING4) has recently been implicated as a repressor of angiogenesis and tumor growth through association with NF-kappaB [6].
One clone, ChET 4, corresponds to the promoter region for beclin 1, a candidate tumor suppressor protein [7].
Candidate tumor-suppressor genes on chromosome arm 8p in early-onset and high-grade breast cancers [8].
Candidate tumor suppressor LUCA-15/RBM5/H37 modulates expression of apoptosis and cell cycle genes [9].
Candidate tumor suppressor genes at FRA7G are coamplified with MET and do not suppress malignancy in a gastric cancer [10].

Biological context of PDSS2

The highly conserved zinc-finger protein, CTCF, is a candidate tumor suppressor protein that binds to highly divergent DNA sequences [11].

Physical interactions of PDSS2

BAP1, a candidate tumor suppressor protein that interacts with BRCA1 [12].

Other interactions of PDSS2

NM23 is a candidate tumor suppressor protein and has recently been identified as an NDP kinase [13].
Candidate tumor-suppressor gene DLEC1 is frequently downregulated by promoter hypermethylation and histone hypoacetylation in human epithelial ovarian cancer [14].
Genetic and Epigenetic Alterations of DLC-1, a Candidate Tumor Suppressor Gene, in Nasopharyngeal Carcinoma [15].

References

Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations. L??pez, L.C., Schuelke, M., Quinzii, C.M., Kanki, T., Rodenburg, R.J., Naini, A., Dimauro, S., Hirano, M. Am. J. Hum. Genet. (2006) [Pubmed]
Link of the unique oncogenic properties of adenovirus type 9 E4-ORF1 to a select interaction with the candidate tumor suppressor protein ZO-2. Glaunsinger, B.A., Weiss, R.S., Lee, S.S., Javier, R. EMBO J. (2001) [Pubmed]
Candidate tumor suppressor HYAL2 is a glycosylphosphatidylinositol (GPI)-anchored cell-surface receptor for jaagsiekte sheep retrovirus, the envelope protein of which mediates oncogenic transformation. Rai, S.K., Duh, F.M., Vigdorovich, V., Danilkovitch-Miagkova, A., Lerman, M.I., Miller, A.D. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
Distinct regions of allelic loss on 13q in prostate cancer. Cooney, K.A., Wetzel, J.C., Merajver, S.D., Macoska, J.A., Singleton, T.P., Wojno, K.J. Cancer Res. (1996) [Pubmed]
Deletion mapping using quantitative real-time PCR identifies two distinct 3p21.3 regions affected in most cervical carcinomas. Senchenko, V., Liu, J., Braga, E., Mazurenko, N., Loginov, W., Seryogin, Y., Bazov, I., Protopopov, A., Kisseljov, F.L., Kashuba, V., Lerman, M.I., Klein, G., Zabarovsky, E.R. Oncogene (2003) [Pubmed]
The candidate tumor suppressor ING4 represses activation of the hypoxia inducible factor (HIF). Ozer, A., Wu, L.C., Bruick, R.K. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
Use of chromatin immunoprecipitation to clone novel E2F target promoters. Weinmann, A.S., Bartley, S.M., Zhang, T., Zhang, M.Q., Farnham, P.J. Mol. Cell. Biol. (2001) [Pubmed]
Candidate tumor-suppressor genes on chromosome arm 8p in early-onset and high-grade breast cancers. Armes, J.E., Hammet, F., de Silva, M., Ciciulla, J., Ramus, S.J., Soo, W.K., Mahoney, A., Yarovaya, N., Henderson, M.A., Gish, K., Hutchins, A.M., Price, G.R., Venter, D.J. Oncogene (2004) [Pubmed]
Candidate tumor suppressor LUCA-15/RBM5/H37 modulates expression of apoptosis and cell cycle genes. Mourtada-Maarabouni, M., Keen, J., Clark, J., Cooper, C.S., Williams, G.T. Exp. Cell Res. (2006) [Pubmed]
Candidate tumor suppressor genes at FRA7G are coamplified with MET and do not suppress malignancy in a gastric cancer. Han, S.Y., Druck, T., Huebner, K. Genomics (2003) [Pubmed]
Transcriptional repression by the insulator protein CTCF involves histone deacetylases. Lutz, M., Burke, L.J., Barreto, G., Goeman, F., Greb, H., Arnold, R., Schultheiss, H., Brehm, A., Kouzarides, T., Lobanenkov, V., Renkawitz, R. Nucleic Acids Res. (2000) [Pubmed]
BAP1, a candidate tumor suppressor protein that interacts with BRCA1. Jensen, D.E., Rauscher, F.J. Ann. N. Y. Acad. Sci. (1999) [Pubmed]
Localization of a second NM23 gene, NME2, to chromosome 17q21-q22. Kelsell, D.P., Black, D.M., Solomon, E., Spurr, N.K. Genomics (1993) [Pubmed]
Candidate tumor-suppressor gene DLEC1 is frequently downregulated by promoter hypermethylation and histone hypoacetylation in human epithelial ovarian cancer. Kwong, J., Lee, J.Y., Wong, K.K., Zhou, X., Wong, D.T., Lo, K.W., Welch, W.R., Berkowitz, R.S., Mok, S.C. Neoplasia (2006) [Pubmed]
Genetic and Epigenetic Alterations of DLC-1, a Candidate Tumor Suppressor Gene, in Nasopharyngeal Carcinoma. Peng, D., Ren, C.P., Yi, H.M., Zhou, L., Yang, X.Y., Li, H., Yao, K.T. Acta Biochim. Biophys. Sin. (Shanghai) (2006) [Pubmed]

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