BCS1L and RPE

		BCS1L	RPE
Description		BCS1 ubiquinol-cytochrome c reductase complex chaperone	ribulose-5-phosphate-3-epimerase
Image		No pdb structure
GO Annotations	Cellular Component	Mitochondrion Mitochondrial Inner Membrane Membrane Respiratory Chain Complex III	Cytosol Extracellular Exosome
	Molecular Function	Nucleotide Binding Protein Binding ATP Binding Hydrolase Activity ATP Hydrolysis Activity	D-ribulose-phosphate 3-epimerase Activity Protein Binding Isomerase Activity Racemase And Epimerase Activity, Acting On Carbohydrates And Derivatives Identical Protein Binding Protein Homodimerization Activity Metal Ion Binding
	Biological Process	Mitochondrion Organization Protein Insertion Into Mitochondrial Inner Membrane From Matrix Mitochondrial Respiratory Chain Complex I Assembly Mitochondrial Cytochrome C Oxidase Assembly Mitochondrial Respiratory Chain Complex III Assembly	Carbohydrate Metabolic Process Pentose-phosphate Shunt Pentose-phosphate Shunt, Non-oxidative Branch
Pathways		Mitochondrial protein import Complex III assembly	Pentose phosphate pathway
Drugs
Diseases		Bjornstad syndrome Mitochondrial respiratory chain deficiencies (MRCD), including: Mitochondrial complex I deficiency (MT-C1D); Complex II deficiency (MT-C2D); Complex III deficiency (MT-C3D); Complex IV deficiency (MT-C4D); Complex V deficiency (MT-ATPSD); Leigh syndrome (LS); Kearns-Sayre Syndrome (KSS); LCHD deficiency (LCHD); Leber Hereditary Optic Neuropathy (LHON); Myoclonic Epilepsy and Ragged-Red Fiber Disease (MERRF); NARP; MELAS; ACAD9 deficiency; HADH deficiency; HIBCH deficiency; GRACILE syndrome
GWAS		Male-pattern baldness ( 28196072)	Serum metabolite levels ( 23093944)
Interacting Genes		3 interacting genes: DDX24 DNAJA1 MTDH	2 interacting genes: EXOSC10 PLEKHF2
Entrez ID		617	6120
HPRD ID		04708	01608
Ensembl ID		ENSG00000074582	ENSG00000197713
Uniprot IDs		A0A024R445 A8JZZ8 Q9Y276	C9IZU8 C9J9T0 Q96AT9
PDB IDs			3OVP 3OVQ 3OVR 3QC3
Enriched GO Terms of Interacting Partners?		Protein Complex Involved In Cell Adhesion	Nuclear Polyadenylation-dependent SnoRNA Catabolic Process Nuclear Polyadenylation-dependent SnRNA Catabolic Process Nuclear Polyadenylation-dependent CUT Catabolic Process Nuclear Polyadenylation-dependent Antisense Transcript Catabolic Process Protein Insertion Into Mitochondrial Inner Membrane From Matrix SnRNA Catabolic Process Sno(s)RNA Catabolic Process Positive Regulation Of MRNA Cis Splicing, Via Spliceosome CUT Catabolic Process Exonucleolytic Trimming To Generate Mature 3'-end Of 5.8S RRNA From Tricistronic RRNA Transcript (SSU-rRNA, 5.8S RRNA, LSU-rRNA) TRNA Surveillance TRAMP-dependent TRNA Surveillance Pathway Nuclear Polyadenylation-dependent RRNA Catabolic Process Respiratory Chain Complex III Mitochondrial Respiratory Chain Complex III Assembly RRNA 3'-end Processing Poly(A)-dependent SnoRNA 3'-end Processing Nucleolar Exosome (RNase Complex) TRNA Decay Nuclear MRNA Surveillance Regulation Of Telomerase RNA Localization To Cajal Body Cytoplasmic Exosome (RNase Complex) RNA Exonuclease Activity Histone MRNA Catabolic Process Exosome (RNase Complex) Nuclear Exosome (RNase Complex) Sno(s)RNA Metabolic Process 3'-5' Exonuclease Activity Protein Insertion Into Mitochondrial Inner Membrane Maturation Of 5.8S RRNA Mitochondrial Cytochrome C Oxidase Assembly Negative Regulation Of Telomere Maintenance Via Telomerase Telomerase RNA Binding Respiratory Chain Complex IV Assembly Histone MRNA Metabolic Process Positive Regulation Of MRNA Splicing, Via Spliceosome 3'-5'-RNA Exonuclease Activity Negative Regulation Of DNA Biosynthetic Process Negative Regulation Of Telomere Maintenance Via Telomere Lengthening Protein Insertion Into Mitochondrial Membrane Establishment Of Protein Localization To Mitochondrial Membrane NADH Dehydrogenase Complex Assembly Inner Mitochondrial Membrane Organization Mitochondrial Respiratory Chain Complex I Assembly Cytochrome Complex Assembly Negative Regulation Of Telomere Maintenance Nuclear RNA Surveillance Nuclear-transcribed MRNA Catabolic Process, Nonsense-mediated Decay RNA Surveillance Positive Regulation Of RNA Splicing
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