IMMP2L Human

IMP2 Inner Mitochondrial Membrane Peptidase-Like Human Recombinant
Cat. No.
BT22777
Source
Escherichia Coli.
Synonyms
IMP2 Inner Mitochondrial Membrane Peptidase-Like (S. Cerevisiae), IMP2, IMP2 Inner Mitochondrial Membrane Protease-Like (S. Cerevisiae), IMMP2L Intronic Transcript 1 (Non-Protein Coding), Mitochondrial Inner Membrane Protease Subunit, Inner Mitochondrial Membrane Peptidase 2 Like, IMP2-Like Protein, EC 3.4.21.- , IMMP2L-IT1, IMP2-LIKE, EC 3.4.21, Mitochondrial inner membrane protease subunit 2.
Appearance
Sterile filtered colorless solution.
Purity
Greater than 95.0% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. They may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description

IMMP2L Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 161 amino acids (38-175aa) and having a molecular mass of 18.0kDa. 
IMMP2L is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

Product Specs

Introduction
IMP2 Inner Mitochondrial Membrane Peptidase-Like (IMMP2L) is implicated in processing the signal peptide sequences. IMMP2L is used to direct mitochondrial proteins to the mitochondria. IMMP2L resides in the mitochondria and is one of the essential proteins for the catalytic activity of the mitochondrial inner membrane peptidase (IMP) complex. Two variants which encode the same protein have been found for IMMP2L.
Description
IMMP2L Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 161 amino acids (38-175aa) and having a molecular mass of 18.0kDa. IMMP2L is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
Physical Appearance
Sterile filtered colorless solution.
Formulation
IMMP2L protein solution (0.5mg/ml) containing 20mM Tris-HCl buffer (pH 8.0), 0.15M NaCl, 50% glycerol and 1mM DTT.
Stability
Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.
Purity
Greater than 95.0% as determined by SDS-PAGE.
Synonyms
IMP2 Inner Mitochondrial Membrane Peptidase-Like (S. Cerevisiae), IMP2, IMP2 Inner Mitochondrial Membrane Protease-Like (S. Cerevisiae), IMMP2L Intronic Transcript 1 (Non-Protein Coding), Mitochondrial Inner Membrane Protease Subunit, Inner Mitochondrial Membrane Peptidase 2 Like, IMP2-Like Protein, EC 3.4.21.- , IMMP2L-IT1, IMP2-LIKE, EC 3.4.21, Mitochondrial inner membrane protease subunit 2.
Source
Escherichia Coli.
Amino Acid Sequence
MGSSHHHHHH SSGLVPRGSH MGSRVEGASM QPSLNPGGSQ SSDVVLLNHW KVRNFEVHRG DIVSLVSPKN PEQKIIKRVI ALEGDIVRTI GHKNRYVKVP RGHIWVEGDH HGHSFDSNSF GPVSLGLLHA HATHILWPPE RWQKLESVLP PERLPVQREE E.

Product Science Overview

Gene and Protein Structure

The IMMP2L gene is located on chromosome 7 at the band 7q31.1 and consists of 18 exons . The gene encodes for the inner mitochondrial membrane peptidase subunit 2, which is an enzyme involved in the removal of transit peptides. These peptides are essential for targeting proteins from the mitochondrial matrix, across the inner membrane, into the intermembrane space .

The IMMP2L protein has two isoforms due to alternative splicing. One isoform is composed of 175 amino acids with a molecular weight of approximately 19.7 kDa, while the other isoform consists of 110 amino acids with a molecular weight of around 12.3 kDa .

Function

IMMP2L is a part of the mitochondrial inner membrane peptidase (IMP) complex, which is essential for the catalytic activity required to process mitochondrial proteins . The primary function of IMMP2L is to catalyze the removal of transit peptides from precursor proteins, facilitating their proper localization within the mitochondria . One of the known substrates processed by IMMP2L is the nuclear-encoded protein DIABLO .

Clinical Significance

Mutations or dysregulation of the IMMP2L gene have been associated with various disorders. Notably, IMMP2L has been linked to Autism Spectrum Disorder and Dystonia 7, Torsion . The protein’s role in mitochondrial function underscores its importance in cellular energy metabolism and overall cellular health.

Research and Applications

Recombinant forms of IMMP2L are used in research to study mitochondrial protein processing and the role of mitochondrial dysfunction in various diseases. Understanding the function and regulation of IMMP2L can provide insights into the development of therapeutic strategies for mitochondrial-related disorders.

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