LONM rabbit pAb

This gene encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. This protein mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides in the mitochondrial matrix. It may also have a chaperone function in the assembly of inner membrane protein complexes, and participate in the regulation of mitochondrial gene expression and maintenance of the integrity of the mitochondrial genome. Decreased expression of this gene has been noted in a patient with hereditary spastic paraplegia (PMID: 18378094). Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2013],

Product Specifications

Background

This gene encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. This protein mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides in the mitochondrial matrix. It may also have a chaperone function in the assembly of inner membrane protein complexes, and participate in the regulation of mitochondrial gene expression and maintenance of the integrity of the mitochondrial genome. Decreased expression of this gene has been noted in a patient with hereditary spastic paraplegia (PMID:18378094) . Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2013]

Product Name Alternative

Lon protease homolog, mitochondrial (EC 3.4.21.-) (LONHs) (Lon protease-like protein) (LONP) (Mitochondrial ATP-dependent protease Lon) (Serine protease 15)

UniProt

P36776

Swiss Prot

P36776

Reactivity

Human; Mouse; Rat

Immunogen

Synthesized peptide derived from human LONM AA range: 398-448

Target

LONM

Clonality

Polyclonal

Source

Rabbit

Applications

Concentration

1 mg/ml

Dilution

WB 1:500-2000

Buffer

-20°C/1 year

Molecular Weight

105kD

Storage Conditions

-20°C/1 year

Observed Molecular Weight

105kD

Fragment

IgG

Subcellular Location

Mitochondrion matrix .

Other Product Names

Lon protease homolog, mitochondrial (EC 3.4.21.-) (LONHs) (Lon protease-like protein) (LONP) (Mitochondrial ATP-dependent protease Lon) (Serine protease 15)