REP2, a protein that is 75% identical and 90% similar to REP1, is able to significantly compensate for the loss of REP1 outside the eye. It is thought that REP2 is not able to fully compensate for the loss of REP1 in the retina. RAB27A, a Rab that has essential functions in the retina, has been shown to be preferentially prenylated by REP1. Additionally, the Rab27a-REP1 and Rab27a-REP2 complexes have different affinities for the Rab geranylgeranyltransferase enzyme, possibly explaining REP2s inability to fully compensate for REP1 in the retina. Culture
A number of individuals in public roles are living with choroidermia, and some have been involved in fundraising efforts for the disease. The former UK Labour Member of Parliament Siôn Simon is a known to have the condition. Comic and activist E.J. Scott, partner of Daredevil actress Deborah Ann Woll, also has choroideremia, and is involved in regular fundraising efforts. References
Further reading
Danny Boren, 2015, "First U.S. Gene Therapy Clinical Trial to treat Choroideremia initiated in Philadelphia," Choroideremia Research Foundation (online), Press Release Summary, January 20, 2015, see [10] Archived 2015-09-21 at the Wayback Machine, accessed 23 April 2015. Cory MacDonald, 2015, "Nightstar Receives U.S. and European Orphan Drug Designation for Gene Therapy to Treat Choroideremia," Choroideremia Research Foundation (online), Press Release Summary, March 24, 2015 (release date, January 11), see [11], accessed 23 April 2015. FFB, 2015, "U.S. Human Study for Choroideremia Gene Therapy Launched by Spark Therapeutics," Foundation Fighting Blindness (online), January 27, 2015, see [12], accessed 23 April 2015.
Benzylpenicillin, also known as penicillin G (PenG) or BENPEN, and in military slang "Peanut Butter Shot" is an antibiotic used to treat a number of bacterial infections. This includes pneumonia, strep throat, syphilis, necrotizing enterocolitis, diphtheria, gas gangrene, leptospirosis, cellulitis, and tetanus. It is not a first-line agent for pneumococcal meningitis. Benzylpenicillin is given by injection into a vein or muscle. Two long-acting forms benzathine benzylpenicillin and procaine benzylpenicillin are available for use by injection into a muscle.Side effects include diarrhea, seizures, and allergic reactions including anaphylaxis. When used to treat syphilis or Lyme disease a reaction known as Jarisch–Herxheimer may occur. It is not recommended in those with a history of penicillin allergy. Use during pregnancy is generally safe in the penicillin and β-lactam class of medications.Benzylpenicillin is on the World Health Organizations List of Essential Medicines. Medical uses
Antimicrobial potency
As an antibiotic, benzylpenicillin is noted to possess effectiveness mainly against gram-positive organisms. Some gram-negative organisms such as Neisseria gonorrhoeae and Leptospira weilii are also reported to be susceptible to benzylpenicillin. Adverse effects
Adverse effects can include hypersensitivity reactions including urticaria, fever, joint pains, rashes, angioedema, anaphylaxis, serum sickness-like reaction. Rarely CNS toxicity including convulsions (especially with high doses or in severe renal impairment), interstitial nephritis, haemolytic anaemia, leucopenia, thrombocytopenia, and coagulation disorders. Also reported diarrhoea (including antibiotic-associated colitis). Benzylpenicillin serum concentrations can be monitored either by traditional microbiological assay or by more modern chromatographic techniques.