SAB1411647

Anti-LONP1 antibody produced in rabbit

purified immunoglobulin, buffered aqueous solution

• Synonym(s): LON, LONP, LonHS, MGC1498, PIM1, PRSS15, hLON
• UNSPSC Code: 12352203
• NACRES: NA.41

Properties

• biological source: rabbit
• Quality Level: 100
• conjugate: unconjugated
• antibody form: purified immunoglobulin
• antibody product type: primary antibodies
• clone: polyclonal
• form: buffered aqueous solution
• mol wt: antigen 106.5 kDa
• species reactivity: human, mouse
• technique(s): immunofluorescence: suitable; western blot: 1 μg/mL
• NCBI accession no.: NM_004793.2
• UniProt accession no.: P36776
• shipped in: dry ice
• storage temp.: −20°C
• target post-translational modification: unmodified
• Gene Information: human ... LONP1(9361)

Description

General description

Lon peptidase 1, mitochondrial (LONP1) is an ATP-dependent mitochondrial Lon protease, encoded by the gene mapped to human chromosome 19p13.3. The encoded protein is a member of the AAA+ superfamily of ATPases.

Immunogen

LONP1 (NP_004784.2, 1 a.a. ~ 959 a.a) full-length human protein.

Sequence
MAASTGYVRLWGAARCWVLRRPMLAAAGGRVPTAAGAWLLRGQRTCDASPPWALWGRGPAIGGQWRGFWEASSRGGGAFSGGEDASEGGAEEGAGGAGGSAGAGEGPVITALTPMTIPDVFPHLPLIAITRNPVFPRFIKIIEVKNKKLVELLRRKVRLAQPYVGVFLKRDDSNESDVVESLDEIYHTGTFAQIHEMQDLGDKLRMIVMGHRRVHISRQLEVEPEEPEAENKHKPRRKSKRGKKEAEDELSARHPAELAMEPTPELPAEVLMVEVENVVHEDFQVTEEVKALTAEIVKTIRDIIALNPLYRESVLQMMQAGQRVVDNPIYLSDMGAALTGAESHELQDVLEETNIPKRLYKALSLLKKEFELSKLQQRLGREVEEKIKQTHRKYLLQEQLKIIKKELGLEKDDKDAIEEKFRERLKELVVPKHVMDVVDEELSKLGLLDNHSSEFNVTRNYLDWLTSIPWGKYSNENLDLARAQAVLEEDHYGMEDVKKRILEFIAVSQLRGSTQGKILCFYGPPGVGKTSIARSIARALNREYFRFSVGGMTDVAEIKGHRRTYVGAMPGKIIQCLKKTKTENPLILIDEVDKIGRGYQGDPSSALLELLDPEQNANFLDHYLDVPVDLSKVLFICTANVTDTIPEPLRDRMEMINVSGYVAQEKLAIAERYLVPQARALCGLDESKAKLSSDVLTLLIKQYCRESGVRNLQKQVEKVLRKSAYKIVSGEAESVEVTPENLQDFVGKPVFTVERMYDVTPPGVVMGLAWTAMGGSTLFVETSLRRPQDKDAKGDKDGSLEVTGQLGEVMKESARIAYTFARAFLMQHAPANDYLVTSHIHLHVPEGATPKDGPSAGCTIVTALLSLAMGRPVRQNLAMTGEVSLTGKILPVGGIKEKTIAAKRAGVTCIVLPAENKKDFYDLAAFITEGLEVHFVEHYREIFDIAFPDEQAEALAVER

Biochem/physiol Actions

Lon peptidase 1, mitochondrial (LONP1) is involved in various cellular processes such as DNA replication, membrane fusion, signal transduction and transcriptional regulation. Genetic variation in the gene leads to a multi-system developmental disorder called cerebral, ocular, dental, auricular, skeletal (CODAS) syndrome. Downregulated expression of this gene has been observed in hereditary spastic paraplegia patients.

Physical form

Solution in phosphate buffered saline, pH 7.4

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Safety Information

• Storage Class: 10 - Combustible liquids
• wgk_germany: WGK 3
• flash_point_f: Not applicable
• flash_point_c: Not applicable