Mitofusin 2 Recombinant Rabbit mAb (一抗) - WB | Bioss

bsm-61395R

重组兔单抗

Mitofusin 2 Recombinant Rabbit mAb

线粒体融合蛋白Mfn2重组兔单抗

CMT2A; CMT2A2; CMT2A2A; CMT2A2B; CPRP1; HMSN6A; HSG; MARF; MSL; D630023P19Rik; Fzo; MFN2_HUMAN; MFN2; Transmembrane GTPase MFN2; 3.6.5.-; KIAA0214; MFN2_MOUSE; Hypertension-related protein 1; Mitochondrial assembly regulatory factor (HSG protein); MFN2_RAT; Mitochondrial transmembrane GTPase FZO1A (Protein HSG); Fzo1a; mitofusin 2

Rabbit

A synthesized peptide derived from human Mitofusin 2: 50-100

IgG

Liquid

affinity purified by Protein A

Recombinant

2D17

86 kDa

80 kDa

10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.

Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles.

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

MFN2

Mitofusin-2

Forms homomultimers and heteromultimers with MFN1.

Mitochondrion outer membrane; Multi-pass membrane protein.

Ubiquitous; expressed at low level. Highly expressed in heart and kidney.

Defects in MFN2 are the cause of Charcot-Marie-Tooth disease type 2A2 (CMT2A2) [MIM:609260]. CMT2A2 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Defects in MFN2 are the cause of Charcot-Marie-Tooth disease type 6 (CMT6) [MIM:601152]; also referred to as autosomal dominant hereditary motor and sensory neuropathy VI (HMSN6). CMT6 is an autosomal dominant form of axonal CMT associated with optic atrophy.

Belongs to the mitofusin family.