After showing a change of –0.11 in ONLS score at 15 months, patients in the high-dose PXT3003 group had an additional mean improvement of –0.17 in the following 45 months. The low-dose group didn’t see as much efficacy signal, but still had improvements of –0.17 in ONLS score in the final 45 months of treatment, following a –0.05 change seen at the 15-month mark. Notably, among patients receiving placebo in the original 15-week period who then switched to low-dose or high-dose PXT3003 during period 1 and high dose during period 2, the mean change in ONLS score was –0.11 in the final 45 months compared with a worsening change of 0.17 in the original 15-week treatment period. CMT1A, the most common form of CMT, is caused by a duplication of the PMP22 gene that is responsible for the production of the peripheral myelin sheath protein. Its duplication leads to abnormal levels of PMP22 protein, which then leads to a failure in the production of normal myelin. In data from preclinical studies, PXT3003 was shown to suppress PMP22 production and improve neuromuscular function. Following the data announcement, Shahram Attarian, MD, PhD, head of the Neuromuscular Diseases Service and ALS Department at Timone Hospital in Marseilles, France, said in a statement that “as an investigator involved since the beginning in the clinical development of PXT3003 in CMT1A, I find these long-term safety and efficacy data very encouraging. Being able to stabilize, or even improve, patients with CMT1A is an extremely worthwhile goal, particularly as these individuals will inevitably decline following the long-term natural course of the disease with the currently available standard of care.” 3 Attarian, who served as lead investigator for PLEO-CMT and PLEO-CMT-FU, currently serves as lead investigator of the PREMIER trial. REFERENCES

After running the update press "X" and the custom firmware will install, all been well your PSP will now be running custom firmware until you power it off. Unzip and copy the program to your PSP > Game folder like you did with the custom firmware installer, then load PSP Ident up on your PSP: Mathieulh, Geohotz, TPU and everyone else involved for making PSP signing possible, making the Minna No Sukkiri Exploit redundant. If you are installing ME just run the installer and follow the directions on the display. Once complete you should now have permanent CFW. The open-label extension of that study, PLEO-CMT-FU, had results announced in May 2022, which further confirmed the long-term safety and effectiveness of PXT3003 over a 5-year treatment period. The data readout included patients who had 15 months in PLEO-CMT, followed by 9 months in period 1 of PLEO-CMT-FU and 36 months of period 2 in the same extension study. 3Something to note is that you can only permanently install this firmware on a PSP 1000 or a PSP 2000 that is not an 88 v3 version. However its very easy to re load the custom firmware on a 88V3 PSP 2000, PSP 3000, PSP Go or E1000. I will cover finding out your PSP version later in this guide, all you need to know for now is that if you own a PSP you can follow this guide and get homebrew applications running on your PSP. There are 2 options for permanent CFW for the 2000 model. You can use cIPL flasher or use Infinity 2.0. CIPL flasher was meant for only 1000 and some 2000 models. Please read carefully below on how to determine if cIPL Flasher or Infinity 2.0 should be used.

Note: On the 15/01/2015 Sony released PSP firmware 6.61, some three and a half years after the last 6.60 update. It's of no benefit to you updating to 6.61 after all this time, however the custom firmware has now been updated to support 6.61. So you can now follow this guide if you happen to have updated to 6.61. bbtgp and Draan for their PrxEncrypter source code to produce the signed fake vshmain module for permanent patch Attarian S, Young P, Brannagan TH, et al. A double-blind, placebo-controlled, randomized trial of PXT3003 for the treatment of Charcot-Marie-Tooth type 1A. Orphanet J Rare Dis. 2021;16(1):433. doi:10.1186/s13023-021-02040-8 PXT3003 (PHARNEXT SA), a novel fixed-dose synergistic combination of baclofen, naltrexone, and sorbitol, is being evaluated in the pivotal phase 3 PREMIER study (NCT04762758) of patients with Charcot- Marie-Tooth disease type 1a (CMT1A), a neurological disease for which there are no curative or symptomatic medications approved. The international, randomized, double-blind, 2-arm, placebo-controlled trial is taking place in 52 centers across the United States, Canada, Europe, and Israel, with topline data expected to be announced in Q4 2023. 1

VirtuousFlame: For the ISO Loading Code, Custom PSX EBOOT Support and most of the CFW related code. I have personally tested and made this firmware permanent on my PSP 1000 and a friends PSP 2000 (Non 88v3). If you are unsure about anything just use the PROUPDATE and enjoy custom firmware. If you ever power your PSP off fully just use the fast recovery program, there is little risk to your PSP doing this.