Antibody Name:	Anti-LRPPRC Antibody
Antibody SKU:	CAB3365
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1041-1394 of human LRPPRC (NP_573566.2).

Application:	WB IHC IF
Recommended Dilution:	WB 1:1000 - 1:2000 IHC 1:100 - 1:200 IF 1:100 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	HeLa, Mouse liver, Rat lung

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1041-1394 of human LRPPRC (NP_573566.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	IACR LNQK KGAY DIFL NAKE QNIV FNAE TYSN LIKL LMSE DYFT QAME VKAF AETH IKGF TLND AANS RLII TQVR RDYL KEAV TTLK TVLD QQQT PSRL AVTR VIQA LAMK GDVE NIEV VQKM LNGL EDSI GLSK MVFI NNIA LAQI KNNN IDAA IENI ENML TSEN KVIE PQYF GLAY LFRK VIEE QLEP AVEK ISIM AERL ANQF AIYK PVTD FFLQ LVDA GKVD DARA LLQR CGAI AEQT PILL LFLL RNSR KQGK ASTV KSVL ELIP ELNE KEEA YNSL MKSY VSEK DVTS AKAL YEHL TAKN TKLD DLFL KRYA SLLK YAGE PVPF IEPP ESFE FYAQ QLRK LREN SS
Gene ID:	10128
Uniprot:	P42704
Cellular Location:	Mitochondrion, Nucleus, Nucleus inner membrane, Nucleus outer membrane, nucleoplasm
Calculated MW:	157kDa
Observed MW:	130kDa

Synonyms:

LRPPRC, CLONE-23970, GP130, LRP130, LSFC

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.

UniProt Protein Function:	LRPPRC: 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. Defects in LRPPRC are the cause of Leigh syndrome French- Canadian type (LSFC). Leigh syndrome is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency. In the Saguenay-Lac Saint Jean region of Quebec province in Canada, a biochemically distinct form of Leigh syndrome with COX deficiency has been described. Patients have been observed to have a developmental delay, hypotonia, mild facial dysmorphism, chronic well-compensated metabolic acidosis, and high mortality due to episodes of severe acidosis and coma. Enzyme activity was close to normal in kidney and heart, 50% of normal in fibroblasts and skeletal muscle, and nearly absent in brain and liver. LSFC patients show reduced (<30%) levels of LRPPRC in both fibroblast and liver mitochondria and a specifically reduced translation of COX subunits MT-CO1/COXI and MT-CO3 (COXIII).
UniProt Protein Details:	Protein type:RNA-binding Chromosomal Location of Human Ortholog: 2p21 Cellular Component: nucleoplasm; nuclear outer membrane; microtubule; condensed nuclear chromosome; cytoskeleton; mitochondrion; membrane; perinuclear region of cytoplasm; nuclear inner membrane; nucleus; ribonucleoprotein complex Molecular Function:actin filament binding; protein binding; RNA binding; microtubule binding; beta-tubulin binding; single-stranded DNA binding Biological Process: mRNA transport; transcription, DNA-dependent; regulation of transcription, DNA-dependent; mitochondrion transport along microtubule Disease: Leigh Syndrome, French Canadian Type
NCBI Summary:	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. [provided by RefSeq, Mar 2012]
UniProt Code:	P42704
NCBI GenInfo Identifier:	156632706
NCBI Gene ID:	10128
NCBI Accession:	P42704.3
UniProt Related Accession:	P42704
Molecular Weight:	~ 130kDa
NCBI Full Name:	Leucine-rich PPR motif-containing protein, mitochondrial
NCBI Synonym Full Names:	leucine rich pentatricopeptide repeat containing
NCBI Official Symbol:	LRPPRC
NCBI Official Synonym Symbols:	LSFC; GP130; LRP130; CLONE-23970
NCBI Protein Information:	leucine-rich PPR motif-containing protein, mitochondrial
UniProt Protein Name:	Leucine-rich PPR motif-containing protein, mitochondrial
UniProt Synonym Protein Names:	130 kDa leucine-rich protein; LRP 130; GP130
Protein Family:	Leucine-rich PPR motif-containing protein
UniProt Gene Name:	LRPPRC
UniProt Entry Name:	LPPRC_HUMAN

	Western blot analysis of extracts of various cell lines, using LRPPRC antibody (CAB3365) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 5s.
	Immunohistochemistry of paraffin-embedded Human colon using LRPPRC Rabbit pAb (CAB3365) at dilution of 1:100 (40x lens).
	Confocal immunofluorescence analysis of Hela cells using LRPPRC Polyclonal Antibody (CAB3365) at dilution of 1:400. Blue: DAPI for nuclear staining.