Mouse anti-Human LONP1 Monoclonal Antibody | anti-LONP1 antibody

LONP1 (PRSS15, Lon Protease Homolog, Mitochondrial, LONHs, Lon Protease-like Protein, Mitochondrial ATP-dependent Protease Lon, Serine Protease 15)

Cat. Num. AAA6005599

• Gene Names: LONP1; LON; LONP; PIM1; hLON; LonHS; PRSS15
• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: LONP1; Monoclonal Antibody; LONP1 (PRSS15; Lon Protease Homolog; Mitochondrial; LONHs; Lon Protease-like Protein; Mitochondrial ATP-dependent Protease Lon; Serine Protease 15); Anti -LONP1 (PRSS15; anti-LONP1 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 3B2
• Specificity: Recognizes human PRSS15.
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2.
• Sequence: YVAQEKLAIAERYLVPQARALCGLDESKAKLSSDVLTLLIKQYCRESGVRNLQKQVEKVLRKSAYKIVSGEAESVEVTPENLQDFVGKPVFTVERMYDVTP

• Applicable Applications for anti-LONP1 antibody: ELISA (EL/EIA), Western Blot (WB)
• Application Notes: Suitable for use in ELISA and Western Blot.
• Immunogen: Partial recombinant corresponding to aa661-761 from PRSS15 (NP_004784) with GST tag. MW of the GST tag alone is 26kD.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot detection against Immunogen (36.85kD).)

Related Product Information for anti-LONP1 antibody

This gene encodes a mitochondrial matrix protein in the Lon family of ATP-dependent proteases. A similar E. coli protein regulates gene expression by targeting specific regulatory proteins for degradation. This protein binds a specific sequence in the light and heavy chain promoters of the mitochondrial genome which are involved in regulation of DNA replication and transcription.

Product Categories/Family for anti-LONP1 antibody

Antibodies; Abs to Enzymes; Peptidase

NCBI and Uniprot Product Information

• NCBI GI #: 451327636
• NCBI GeneID: 9361
• NCBI Accession #: NP_001263409.1
• NCBI GenBank Nucleotide #: NM_001276480.1
• UniProt Accession #: P36776; P36777; Q9UQ95; D6W635; E5KMH8
• Molecular Weight: 106,489 Da
• NCBI Official Full Name: lon protease homolog, mitochondrial isoform 3
• NCBI Official Synonym Full Names: lon peptidase 1, mitochondrial
• NCBI Official Symbol: LONP1
• NCBI Official Synonym Symbols: LON; LONP; PIM1; hLON; LonHS; PRSS15
• NCBI Protein Information: lon protease homolog, mitochondrial; serine protease 15; hLON ATP-dependent protease; mitochondrial lon protease-like protein; mitochondrial ATP-dependent protease Lon
• UniProt Protein Name: Lon protease homolog, mitochondrial
• UniProt Gene Name: LONP1
• UniProt Synonym Gene Names: PRSS15; LONP
• UniProt Entry Name: LONM_HUMAN

NCBI Description

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]

Uniprot Description

PRSS15: ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single- stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site- specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters. Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein. Belongs to the peptidase S16 family.

Protein type: Mitochondrial; DNA-binding; EC 3.4.21.-; RNA-binding; Protease

Chromosomal Location of Human Ortholog: 19p13.2

Cellular Component: mitochondrion; membrane; mitochondrial matrix; cytoplasm

Molecular Function: G-quadruplex DNA binding; protein binding; single-stranded RNA binding; sequence-specific DNA binding; ATP-dependent peptidase activity; serine-type endopeptidase activity; ADP binding; single-stranded DNA binding; ATP binding

Biological Process: mitochondrion organization and biogenesis; mitochondrial genome maintenance; proteolysis involved in cellular protein catabolic process; misfolded or incompletely synthesized protein catabolic process; response to hypoxia; response to hormone stimulus; chaperone-mediated protein complex assembly; mitochondrial DNA metabolic process; protein homooligomerization; aging; response to aluminum ion

Disease: Codas Syndrome