Recombinant Human Leucine-rich PPR motif-containing protein, mitochondrial (LRPPRC), Truncated

Cat.No	ACP23612	Target Name	LRPPRC
Target Synonyms	130 kDa leucine-rich protein; gp130; Leucine-rich PPR motif-containing protein; Leucine-rich PPR-motif containing protein ; LPPRC_HUMAN; LRP 130; lrpprc; mitochondrial	Form	Lyophilized powder
Expression System	Custom Production. Please inquire and provide the desire expression system.	Protein Length	Partial
Purity	>85% (SDS-PAGE)	Storage Buffer	5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Cat.No

ACP23612

Target Name

LRPPRC

Target Synonyms

130 kDa leucine-rich protein; gp130; Leucine-rich PPR motif-containing protein; Leucine-rich PPR-motif containing protein ; LPPRC_HUMAN; LRP 130; lrpprc; mitochondrial

Form

Lyophilized powder

Expression System

Custom Production. Please inquire and provide the desire expression system.

Protein Length

Partial

Purity

>85% (SDS-PAGE)

Storage Buffer

5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Target Species	Human	Uniprot ID	P42704

Background Information

Uniprot Id

P42704
Target Species

Human
Target Name

LRPPRC
Target Full Name

Leucine-rich PPR motif-containing protein, mitochondrial
Target Function

May play a role in RNA metabolism in both nuclei and mitochondria. In the nucleus binds to HNRPA1-associated poly(A) mRNAs and is part of nmRNP complexes at late stages of mRNA maturation which are possibly associated with nuclear mRNA export. May bind mature mRNA in the nucleus outer membrane. In mitochondria binds to poly(A) mRNA. Plays a role in translation or stability of mitochondrially encoded cytochrome c oxidase (COX) subunits. May be involved in transcription regulation. Cooperates with PPARGC1A to regulate certain mitochondrially encoded genes and gluconeogenic genes and may regulate docking of PPARGC1A to transcription factors. Seems to be involved in the transcription regulation of the multidrug-related genes MDR1 and MVP. Part of a nuclear factor that binds to the invMED1 element of MDR1 and MVP gene promoters. Binds single-stranded DNA.
Target Involvement

Leigh syndrome French-Canadian type (LSFC)
Target Subcellular Location

Mitochondrion. Nucleus, nucleoplasm. Nucleus inner membrane. Nucleus outer membrane. Note=Seems to be predominantly mitochondrial.
Target Tissue Specificity

Expressed ubiquitously. Expression is highest in heart, skeletal muscle, kidney and liver, intermediate in brain, non-mucosal colon, spleen and placenta, and lowest in small intestine, thymus, lung and peripheral blood leukocytes.
Target Synonyms

130 kDa leucine-rich protein; gp130; Leucine-rich PPR motif-containing protein; Leucine-rich PPR-motif containing protein ; LPPRC_HUMAN; LRP 130; lrpprc; mitochondrial
Target Background

This gene encodes a leucine-rich protein that has multiple pentatricopeptide repeats (PPR). The precise role of this protein is unknown but studies suggest it may play a role in cytoskeletal organization, vesicular transport, or in transcriptional regulation of both nuclear and mitochondrial genes. The protein localizes primarily to mitochondria and is predicted to have an N-terminal mitochondrial targeting sequence. Mutations in this gene are associated with the French-Canadian type of Leigh syndrome.

Recombinant Human Leucine-rich PPR motif-containing protein, mitochondrial (LRPPRC), Truncated

Specifications

Immunogen Information

Inquire Recombinant Human Leucine-rich PPR motif-containing protein, mitochondrial (LRPPRC), Truncated Now

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