Use my Search Websuite to scan PubMed, PMCentral, Journal Hosts and Journal Archives, FullText. Kick-your-searchterm to multiple Engines kick-your-query now !>

A dictionary by aggregated review articles of nephrology, medicine and the life sciences Your one-stop-run pathway from word to the immediate pdf of peer-reviewed on-topic knowledge.

10.1093/hmg/ddt572 http://scihub22266oqcxt.onion/10.1093/hmg/ddt572 C4014186!4014186 !24334770     free     free     free Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 211.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 245.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 245.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 245.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\24334770 .jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117       Hum+Mol+Genet 2014 ; 23 (11 ): 2791-801 Nephropedia Template TP {{tp|p=24334770 |t=2014. Znē? dyshomeostasis caused by loss of ATP13A2/PARK9 leads to lysosomal dysfunction and alpha-synuclein accumulation |pdf=|usr=}}{{24334770 }} gab.com Text Znē dyshomeostasis caused by loss of ATP13A2/PARK9 leads to lysosomal dysfunction and alpha-synuclein accumulation JO: Hum Mol Genet http://www.kidney.de/pget.php?&tw=1&pmid=24334770 #FOAvip Mutations in ATP13A2 (PARK9) cause Kufor-Rakeb syndrome (KRS) characterized by juvenile-onset parkinsonism, pyramidal signs and dementia. PARK9 belongs to type 5 P-type ATPase with its putative function as a cation transporter. Loss of PARK9 leads to lysosomal dysfunction and subsequent ?-synuclein (?-Syn) accumulation. However, the mechanistic link between PARK9 and lysosomal dysfunction remains unclear. Here, we found that patient fibroblasts expressing mutant PARK9 or primary neurons with silenced PARK9 exhibited increased sensitivity to extracellular zinc (Zn(2+)). This effect was rescued with the Zn(2+) chelators clioquinol or TPEN. PARK9-deficient cells showed decreased lysosomal sequestration of Zn(2+) and increased expression of zinc transporters. Importantly, increased concentrations of Zn(2+) (Zn(2+) stress) resulted in lysosomal dysfunction that was partially restored by expression of wild-type PARK9. Zn(2+) stress also caused increased expression of ?-Syn and consequently decreased activity of the lysosomal enzyme glucocerebrosidase. Together, these data suggest that PARK9 loss of function leads to dyshomeostasis of intracellular Zn(2+) that in turn contributes to lysosomal dysfunction and accumulation of ?-Syn. It will be of interest to examine whether therapeutic lowering of zinc may prove beneficial for patients with KRS. Twit Text FOAVip Znē dyshomeostasis caused by loss of ATP13A2/PARK9 leads to lysosomal dysfunction and alpha-synuclein accumulation ????Hum Mol Genet http://www.kidney.de/pget.php?&tw=1&pmid=24334770 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=24334770 #FOAvip English Wikipedia <ref>{{Cite pmid|24334770 }}</ref> Znē? dyshomeostasis caused by loss of ATP13A2/PARK9 leads to lysosomal dysfunction and alpha-synuclein accumulation #MMPMID24334770 Tsunemi T ; Krainc D Hum Mol Genet 2014[Jun]; 23 (11 ): 2791-801 PMID24334770 show ga Mutations in ATP13A2 (PARK9) cause Kufor-Rakeb syndrome (KRS) characterized by juvenile-onset parkinsonism, pyramidal signs and dementia. PARK9 belongs to type 5 P-type ATPase with its putative function as a cation transporter. Loss of PARK9 leads to lysosomal dysfunction and subsequent ?-synuclein (?-Syn) accumulation. However, the mechanistic link between PARK9 and lysosomal dysfunction remains unclear. Here, we found that patient fibroblasts expressing mutant PARK9 or primary neurons with silenced PARK9 exhibited increased sensitivity to extracellular zinc (Zn(2+)). This effect was rescued with the Zn(2+) chelators clioquinol or TPEN. PARK9-deficient cells showed decreased lysosomal sequestration of Zn(2+) and increased expression of zinc transporters. Importantly, increased concentrations of Zn(2+) (Zn(2+) stress) resulted in lysosomal dysfunction that was partially restored by expression of wild-type PARK9. Zn(2+) stress also caused increased expression of ?-Syn and consequently decreased activity of the lysosomal enzyme glucocerebrosidase. Together, these data suggest that PARK9 loss of function leads to dyshomeostasis of intracellular Zn(2+) that in turn contributes to lysosomal dysfunction and accumulation of ?-Syn. It will be of interest to examine whether therapeutic lowering of zinc may prove beneficial for patients with KRS. |Homeostasis [MESH] |Humans [MESH] |Lysosomes/*enzymology/genetics/metabolism [MESH] |Parkinsonian Disorders/enzymology/genetics/*metabolism [MESH] |Proton-Translocating ATPases/genetics/*metabolism [MESH] |Zinc/*metabolism [MESH]Znē? dyshomeostasis caused by loss of ATP13A2/PARK9 leads to lysosomal(epmc).pdf DeepDyve Pubget Overpricing