Compounds and compositions as channel activating protease inhibitors

A compound of Formula (1):

• or pharmaceutically acceptable salts thereof, wherein
• O—(CR2)p—R2 is a substituent at any position on ring A;
• J is a 5-12 membered monocyclic or fused carbocyclic ring, heterocyclic ring comprising N, O and/or S;
• aryl or heteroaryl ring, provided J is not triazolyl;
• B is
• or (CR2)k—R5;
• Y is a bond, —SO2—, —NHCO— or —O—(CO)—;
• R1 is halo, —(CR2)l—NR6R7, —(CR2)l—NRC(═NR)—NR6R7, —(CR2)l—C(═NR)—NR6R7, —C(O)—(CR2)l—NR6R7, —(CR2)l—NR—SO2R6, —(CR2)l—NR—C(O)—R6, —(CR2)l—SO2NR6R7, or —(CR2)l—OR6, or an optionally substituted C1-6 alkoxy, C1-6 alkyl, C2-6 alkenyl or C2-6 alkynyl;
• or an optionally substituted carbocyclic ring, heterocyclic ring, aryl or heteroaryl;
• R3 is C1-6 alkyl, C2-6 alkenyl, C2-6 alkynyl or —(CR2)l—R5;
• alternatively, NH—Y—R3 together form NH2;
• R2, R4 and R5 are independently an optionally substituted 5-12 membered carbocyclic ring, heterocyclic ring, aryl or heteroaryl;
• or R4 is H, C1-6 alkyl, C2-6 alkenyl, C2-6 alkynyl, or
• wherein P is C or N and ring E together with P form an optionally substituted 5-12 membered monocyclic or fused ring;
• R6 and R7 are independently H, C1-6 alkyl, C2-6 alkenyl, C2-6 alkynyl or —(CR2)l—R5;
• each R is H, or C1-6 alkyl, C2-6 alkenyl, or C2-6 alkynyl;
• l is 0-6;
• k, m, n and p are independently 1-6;
• x is 0-4;
• provided R4 is piperidinyl when NH—Y—R3 together form NH2;
• and
• further provided that R5 is piperidinyl when B is (CR2)k—R5.