Antibody Name:	Anti-LONP1 Antibody
Antibody SKU:	CAB4293
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 120-400 of human LONP1 (NP_004784.2).

Application:	WB IHC
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	A-549, HL-60, LO2, HT-1080, BT-474, Mouse heart, Mouse kidney, Mouse liver, Rat kidney

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 120-400 of human LONP1 (NP_004784.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	VFPH LPLI AITR NPVF PRFI KIIE VKNK KLVE LLRR KVRL AQPY VGVF LKRD DSNE SDVV ESLD EIYH TGTF AQIH EMQD LGDK LRMI VMGH RRVH ISRQ LEVE PEEP EAEN KHKP RRKS KRGK KEAE DELS ARHP AELA MEPT PELP AEVL MVEV ENVV HEDF QVTE EVKA LTAE IVKT IRDI IALN PLYR ESVL QMMQ AGQR VVDN PIYL SDMG AALT GAES HELQ DVLE ETNI PKRL YKAL SLLK KEFE LSKL QQRL GREV EEKI KQTH RKYL LQEQ L
Gene ID:	9361
Uniprot:	P36776
Cellular Location:	Mitochondrion matrix
Calculated MW:	85kDa/100kDa/106kDa
Observed MW:	106kDa

Synonyms:

LONP1, CODASS, LON, LONP, LonHS, PIM1, PRSS15, hLON

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.

UniProt Protein Function:	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.
UniProt Protein Details:	Protein type:RNA-binding; DNA-binding; Mitochondrial; EC 3.4.21.-; 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; ATP binding; single-stranded DNA 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; response to aluminum ion; aging Disease: Codas Syndrome
UniProt Code:	P36776
NCBI GenInfo Identifier:	12644239
NCBI Gene ID:
NCBI Accession:	P36776.2
Molecular Weight:
NCBI Full Name:	Lon protease homolog, mitochondrial
UniProt Protein Name:	Lon protease homolog, mitochondrial
UniProt Synonym Protein Names:	LONHsLon protease-like protein; Mitochondrial ATP-dependent protease Lon; Serine protease 15
UniProt Gene Name:	LONP1
UniProt Entry Name:	LONM_HUMAN

	Western blot analysis of extracts of various cell lines, using LONP1 antibody (CAB4293) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 5s.
	Immunohistochemistry of paraffin-embedded mouse kidney using LONP1 Rabbit pAb (CAB4293) at dilution of 1:100 (40x lens).
	Immunohistochemistry of paraffin-embedded rat kidney using LONP1 Rabbit pAb (CAB4293) at dilution of 1:100 (40x lens).