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Key Documents

SAB1411647

Sigma-Aldrich

Anti-LONP1 antibody produced in rabbit

purified immunoglobulin, buffered aqueous solution

Sinônimo(s):

LON, LONP, LonHS, MGC1498, PIM1, PRSS15, hLON

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About This Item

Código UNSPSC:
12352203
NACRES:
NA.41

fonte biológica

rabbit

conjugado

unconjugated

forma do anticorpo

purified immunoglobulin

tipo de produto de anticorpo

primary antibodies

clone

polyclonal

forma

buffered aqueous solution

peso molecular

antigen 106.5 kDa

reatividade de espécies

human, mouse

técnica(s)

immunofluorescence: suitable
western blot: 1 μg/mL

nº de adesão NCBI

nº de adesão UniProt

Condições de expedição

dry ice

temperatura de armazenamento

−20°C

modificação pós-traducional do alvo

unmodified

Informações sobre genes

human ... LONP1(9361)

Categorias relacionadas

Descrição geral

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.

Imunogênio

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

Sequence
MAASTGYVRLWGAARCWVLRRPMLAAAGGRVPTAAGAWLLRGQRTCDASPPWALWGRGPAIGGQWRGFWEASSRGGGAFSGGEDASEGGAEEGAGGAGGSAGAGEGPVITALTPMTIPDVFPHLPLIAITRNPVFPRFIKIIEVKNKKLVELLRRKVRLAQPYVGVFLKRDDSNESDVVESLDEIYHTGTFAQIHEMQDLGDKLRMIVMGHRRVHISRQLEVEPEEPEAENKHKPRRKSKRGKKEAEDELSARHPAELAMEPTPELPAEVLMVEVENVVHEDFQVTEEVKALTAEIVKTIRDIIALNPLYRESVLQMMQAGQRVVDNPIYLSDMGAALTGAESHELQDVLEETNIPKRLYKALSLLKKEFELSKLQQRLGREVEEKIKQTHRKYLLQEQLKIIKKELGLEKDDKDAIEEKFRERLKELVVPKHVMDVVDEELSKLGLLDNHSSEFNVTRNYLDWLTSIPWGKYSNENLDLARAQAVLEEDHYGMEDVKKRILEFIAVSQLRGSTQGKILCFYGPPGVGKTSIARSIARALNREYFRFSVGGMTDVAEIKGHRRTYVGAMPGKIIQCLKKTKTENPLILIDEVDKIGRGYQGDPSSALLELLDPEQNANFLDHYLDVPVDLSKVLFICTANVTDTIPEPLRDRMEMINVSGYVAQEKLAIAERYLVPQARALCGLDESKAKLSSDVLTLLIKQYCRESGVRNLQKQVEKVLRKSAYKIVSGEAESVEVTPENLQDFVGKPVFTVERMYDVTPPGVVMGLAWTAMGGSTLFVETSLRRPQDKDAKGDKDGSLEVTGQLGEVMKESARIAYTFARAFLMQHAPANDYLVTSHIHLHVPEGATPKDGPSAGCTIVTALLSLAMGRPVRQNLAMTGEVSLTGKILPVGGIKEKTIAAKRAGVTCIVLPAENKKDFYDLAAFITEGLEVHFVEHYREIFDIAFPDEQAEALAVER

Ações bioquímicas/fisiológicas

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.

forma física

Solution in phosphate buffered saline, pH 7.4

Exoneração de responsabilidade

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.

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Código de classe de armazenamento

10 - Combustible liquids

Classe de risco de água (WGK)

WGK 3

Ponto de fulgor (°F)

Not applicable

Ponto de fulgor (°C)

Not applicable


Certificados de análise (COA)

Busque Certificados de análise (COA) digitando o Número do Lote do produto. Os números de lote e remessa podem ser encontrados no rótulo de um produto após a palavra “Lot” ou “Batch”.

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Decreased expression of the mitochondrial matrix proteases Lon and ClpP in cells from a patient with hereditary spastic paraplegia (SPG13).
Hansen J
Neuroscience, 153, 474-482 (2008)
CODAS syndrome is associated with mutations of LONP1, encoding mitochondrial AAA+ Lon protease.
Strauss KA
American Journal of Human Genetics, 96, 121-135 (2015)
The Chromosomal and Functional Clustering of Markedly Divergent Human-Mouse Orthologs Run
Parallel to their Compositional Features
Fuertes M A
Journal of DNA and RNA Research, 1, 31-49 (2016)
Richa Aishwarya et al.
Redox biology, 36, 101660-101660 (2020-08-05)
Mitochondria are highly dynamic organelles that constantly undergo fission and fusion events to adapt to changes in the cellular environment. Aberrant mitochondrial fission has been associated with several types of cardiovascular dysfunction; inhibition of pathologically aberrant mitochondrial fission has been
Kristina Wardelmann et al.
Molecular metabolism, 21, 68-81 (2019-01-24)
Insulin action in the brain controls metabolism and brain function, which is linked to proper mitochondrial function. Conversely, brain insulin resistance associates with mitochondrial stress and metabolic and neurodegenerative diseases. In the present study, we aimed to decipher the impact

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