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LONP1 Products

(Lon Peptidase 1, Mitochondrial (LONP1))

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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].

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Featured LONP1 Categories

LONP1 Antibodies

High quality antibodies with extensive validation data.

LONP1 Proteins

Proteins for various applications incl. WB, ELISA, IF etc.

Recommended LONP1 Antibodies

Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human, Mouse, Rat
Application WB, ELISA, IF (p), IF (cc), IHC (p), IHC (fro)
Validations
  • (5)
Cat. No. ABIN705806
Quantity 100 μL
Datasheet Datasheet
Reactivity Human
Application WB, IF
Validations
  • (5)
Cat. No. ABIN522820
Quantity 100 μg
Datasheet Datasheet
Reactivity Human
Application WB, IHC, IF, IP
Validations
  • (4)
Cat. No. ABIN1680707
Quantity 100 μg
Datasheet Datasheet

Recommended LONP1 ELISA Kits

Product
Reactivity
Analytical Method
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Analytical Method Quantitative Sandwich ELISA
Validations
Cat. No. ABIN6227622
Quantity 96 tests
Datasheet Datasheet

Recommended LONP1 Proteins

Product
Reactivity
Source
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Source Wheat germ
Validations
  • (1)
Cat. No. ABIN1309661
Quantity 10 μg
Datasheet Datasheet
Reactivity Human
Source Wheat germ
Validations
  • (1)
Cat. No. ABIN1309659
Quantity 10 μg
Datasheet Datasheet

Latest Publications for our LONP1 products

Ishikawa, Kobayashi, Yamada, Umehara, Oka, Nakada: "Concentration of mitochondrial DNA mutations by cytoplasmic transfer from platelets to cultured mouse cells." in: PLoS ONE, Vol. 14, Issue 3, pp. e0213283, (2019) (PubMed).

Sekine, Wang, Sideris, Bunker, Zhang, Youle: "Reciprocal Roles of Tom7 and OMA1 during Mitochondrial Import and Activation of PINK1." in: Molecular cell, Vol. 73, Issue 5, pp. 1028-1043.e5, (2019) (PubMed).

Pareek, Thomas, Pallanck: "Loss of the Drosophila m-AAA mitochondrial protease paraplegin results in mitochondrial dysfunction, shortened lifespan, and neuronal and muscular degeneration." in: Cell death & disease, Vol. 9, Issue 3, pp. 304, (2018) (PubMed).

Boutoual, Meseguer, Villarroya, Martín-Hernández, Errami, Martín, Casado, Armengod: "Defects in the mitochondrial-tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARγ-UCP2-AMPK axis." in: Scientific reports, Vol. 8, Issue 1, pp. 1163, (2018) (PubMed).

Pareek, Thomas, Vincow, Morris, Pallanck: "Lon protease inactivation in Drosophila causes unfolded protein stress and inhibition of mitochondrial translation." in: Cell death discovery, Vol. 4, pp. 51, (2018) (PubMed).

Luo, Wang, Hou, Hu, Jia, Qin, Zuo, Liu, Luo, Song, Wang, Pang: "ATP-Dependent Lon Protease Contributes to Helicobacter pylori-Induced Gastric Carcinogenesis." in: Neoplasia (New York, N.Y.), Vol. 18, Issue 4, pp. 242-52, (2016) (PubMed).

Horner, Wilkins, Badil, Iskarpatyoti, Gale: "Proteomic analysis of mitochondrial-associated ER membranes (MAM) during RNA virus infection reveals dynamic changes in protein and organelle trafficking." in: PLoS ONE, Vol. 10, Issue 3, pp. e0117963, (2015) (PubMed).

Thomas, Andrews, Burman, Lin, Pallanck: "PINK1-Parkin pathway activity is regulated by degradation of PINK1 in the mitochondrial matrix." in: PLoS genetics, Vol. 10, Issue 5, pp. e1004279, (2014) (PubMed).

Jin, Youle: "The accumulation of misfolded proteins in the mitochondrial matrix is sensed by PINK1 to induce PARK2/Parkin-mediated mitophagy of polarized mitochondria." in: Autophagy, Vol. 9, Issue 11, pp. 1750-7, (2013) (PubMed).

Merra, Lago, Ricci, Antuzzi, Gasbarrini, Gasbarrini, Ghirlanda: "Splenomegaly as a primary manifestation of Gaucher disease in a young adult woman." in: Case reports in gastroenterology, Vol. 2, Issue 3, pp. 474-8, (2011) (PubMed).

Synonyms and alternative names related to LONP1

lon peptidase 1, mitochondrial (LONP1), lon peptidase 1, mitochondrial (lonp1), lon peptidase 1, mitochondrial (Lonp1), ATP-dependent Lon protease (PIM1), 1200017E13Rik, fc64d11, hLON, LON, Lon, LonHS, LONP, PIM1, PRSS15, prss15, Prss15, wu:fc64d11

Protein level used designations for LONP1

  • lon peptidase 1, mitochondrial
  • protease, serine, 15
  • lon protease homolog, mitochondrial-like
  • hLON ATP-dependent protease
  • lon protease homolog, mitochondrial
  • mitochondrial ATP-dependent protease Lon
  • mitochondrial lon protease-like protein
  • serine protease 15
  • LONP
  • lon protease-like protein
  • ATP-dependent Lon protease
  • hypothetical protein
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