SCP2 - sterol carrier protein 2 Gene

Also Known as NLTP; SCOX; SCPX; SCP-2; SCP-X; NSL-TP; SCP-CHI

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 6342

About SCP2

Cytogenetic location: 1p32.3 Genomic coordinates (GRCh38): 1:52,927,276-53,051,698 (from NCBI)

This gene has 16 transcripts (splice variants), 259 orthologues, 1 paralogue and is associated with 2 phenotypes. Ubiquitous expression in liver (RPKM 77.0), duodenum (RPKM 30.8) and 24 other tissues.

Summary

This gene encodes two proteins: sterol carrier protein X (SCPx) and sterol carrier protein 2 (SCP2), as a result of transcription initiation from 2 independently regulated promoters. The transcript initiated from the proximal promoter encodes the longer SCPx protein, and the transcript initiated from the distal promoter encodes the shorter SCP2 protein, with the 2 proteins sharing a common C-terminus. Evidence suggests that the SCPx protein is a peroxisome-associated thiolase that is involved in the oxidation of branched chain fatty acids, while the SCP2 protein is thought to be an intracellular lipid transfer protein. This gene is highly expressed in organs involved in lipid metabolism, and may play a role in Zellweger syndrome, in which cells are deficient in peroxisomes and have impaired bile acid synthesis. Alternative splicing of this gene produces multiple transcript variants, some encoding different isoforms.[provided by RefSeq, Aug 2010]

SCP2 Products (9)

mRNA	Protein	Name
NM_001007098.3	NP_001007099.1	sterol carrier protein 2 isoform 2
NM_001007099.3	NP_001007100.1	sterol carrier protein 2 isoform 5 precursor
NM_001007100.3	NP_001007101.1	sterol carrier protein 2 isoform 4
NM_001007250.3	NP_001007251.1	sterol carrier protein 2 isoform 3
NM_001193599.2	NP_001180528.1	sterol carrier protein 2 isoform 7
NM_001193600.2	NP_001180529.1	sterol carrier protein 2 isoform 6
NM_001193617.2	NP_001180546.1	sterol carrier protein 2 isoform 8
NM_001330587.2	NP_001317516.1	sterol carrier protein 2 isoform 9
NM_002979.5	NP_002970.2	sterol carrier protein 2 isoform 1 precursor

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
enables cholesterol binding	IDA IDA: Inferred from direct assay	18465878	GOA
enables cholesterol transfer activity	IMP IMP: Inferred from mutant phenotype	7642518	GOA
enables fatty-acyl-CoA binding	IDA IDA: Inferred from direct assay	18465878	GOA
enables long-chain fatty acyl-CoA binding	IDA IDA: Inferred from direct assay	17418802	GOA
enables oleic acid binding	IDA IDA: Inferred from direct assay	18465878	GOA
enables phosphatidylinositol transfer activity	IDA IDA: Inferred from direct assay	12641450	GOA
enables propionyl-CoA C2-trimethyltridecanoyltransferase activity	EXP EXP: Inferred from Experiment	9245689	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	15182174	GOA
enables signaling receptor binding	IPI IPI: Inferred from physical interaction	21375735	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in inositol trisphosphate biosynthetic process	IDA IDA: Inferred from direct assay	12641450	GOA
involved in intracellular cholesterol transport	IMP IMP: Inferred from mutant phenotype	7642518	GOA
involved in lipid hydroperoxide transport	IDA IDA: Inferred from direct assay	15449949	GOA
involved in phospholipid transport	IDA IDA: Inferred from direct assay	12641450	GOA
involved in positive regulation of intracellular cholesterol transport	IDA IDA: Inferred from direct assay	15449949	GOA
involved in positive regulation of steroid metabolic process	IDA IDA: Inferred from direct assay	1703300	GOA
involved in progesterone biosynthetic process	IDA IDA: Inferred from direct assay	1703300	GOA
involved in protein localization to plasma membrane	IDA IDA: Inferred from direct assay	12641450	GOA
involved in steroid biosynthetic process	IDA IDA: Inferred from direct assay	1703300	GOA

Cellular Component GO Annotation	Evidence	References	Source
located in peroxisome	IDA IDA: Inferred from direct assay	1347505	GOA
part of protein-containing complex	IDA IDA: Inferred from direct assay	19584060	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

SCP2 Protein Structure

Thiolase_N

Thiolase_C

SCP2

0
100
200
300
400
500
547 a.a.

Protein Preferred Names	Protein Names
sterol carrier protein 2	SCP-2/3-oxoacyl-CoA thiolase

SCP2 Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Cross	SCP2	P22307	Cav1	Mus musculus	P49817	Anti Bait CoIP	15182174
Intra	SCP2	P22307	TRIP13	Homo sapiens	Q15645	Y2H Prey Pooling	25416956
Intra	SCP2	P22307	TRIP13	Homo sapiens	Q15645	Validated Y2H	25416956
Intra	SCP2	P22307	TRIP13	Homo sapiens	Q15645	Y2H Array	25416956
Intra	SCP2	P22307	CAV1	Homo sapiens	Q03135	Y2H	15182174

Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases	Alias
Leukoencephalopathy With Dystonia And Motor Neuropathy	Sterol Carrier Protein 2 Deficiency LKDMN Leukoencephalopathy-Dystonia-Motor Neuropathy Syndrome Leukoencephalopathy - Dystonia - Motor Neuropathy
D-Bifunctional Protein Deficiency	Bifunctional Peroxisomal Enzyme Deficiency 17-Beta-Hydroxysteroid Dehydrogenase Iv Deficiency Dbp Deficiency Peroxisomal Bifunctional Enzyme Deficiency Pbfe Deficiency Bifunctional Enzyme Deficiency Pseudo-Zellweger Syndrome Zellweger-Like Syndrome DBPD Protein Deficiency, D-Bifunctional
Rhizomelic Chondrodysplasia Punctata, Type 1	Rhizomelic Chondrodysplasia Punctata Type 1 RCDP1 Peroxisome Biogenesis Disorder 9 Pbd9 Chondrodysplasia Punctata, Rhizomelic Form Cdpr Chondrodystrophia Calcificans Punctata Rhizomelic Chondrodysplasia Punctata 1 Chondrodysplasia Punctata, Rhizomelic, Type 1 Chondrodysplasia Punctata, Rhizomelic
Zellweger Syndrome	Cerebrohepatorenal Syndrome Zellweger Leukodystrophy Zs Congenital Iron Overload Chr Zws Severe Pbd-Zsd Severe Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder
Peach Allergy	Prunus Persica Fruit Allergy
Cherry Allergy	Prunus Avium Fruit Allergy
Vegetable Allergy
Celery Allergy
Yellow Fever	Urban Yellow Fever Jungle Yellow Fever Sylvatic Yellow Fever Yf Yellow Fever, Sylvan Bronze John Yellow Jack Yf- [Yellow Fever] Febris Flava
Apple Allergy	Malus Domestica Fruit Allergy
Food Allergy	Food Hypersensitivity Food Allergies Allergy Food Food Allergen-Induced Hypersensitivity
Nut Allergy	Nut Allergic Reaction Nut Hypersensitivity Allergy To Nuts Allergy Nut
Fruit Allergy	Allergy To Fruit
Pollen Allergy	Hay Fever
Alpha-Methylacyl-Coa Racemase Deficiency	Amacr Deficiency AMACRD
Wheat Allergy	Wheat Allergic Reaction Wheat Hypersensitivity Allergy To Wheat Allergy Wheat
Torticollis	Contracture Of Neck Wry Neck Wry Neck/Torticollis
Orange Allergy	Citrus Sinensis Fruit Allergy
Crustacean Allergy
Refsum Disease, Classic	Refsum Disease Heredopathia Atactica Polyneuritiformis Phytanic Acid Oxidase Deficiency Hmsn Iv Refsum Disease, Adult, 1 Refsum'S Disease Phytanic Acid Storage Disease Hereditary Motor And Sensory Neuropathy Iv Hmsn4 Hmsn Type Iv Hmsn 4 Adult Refsum Disease Classic Refsum Disease Hereditary Motor And Sensory Neuropathy Type Iv Refsum Syndrome Hsmn Iv Disorder Of Cornification 11 Doc 11 Hereditary Sensory And Motor Neuropathy Type 4 Hypertrophic Neuropathy Of Refsum Ard Crd Hereditary Motor And Sensory Neuropathy Type 4 Phytanic-Coa Hydroxylase Deficiency RD
Chlorhexidine Allergy
Shrimp Allergy
Dystonia	Dystonic Disease Dystonic Disorder Dystonia Disorders Neuroleptic Dyskinesia
Neonatal Adrenoleukodystrophy	Nald Adrenoleukodystrophy Autosomal Neonatal Form Intermediate Pbd-Zsd Intermediate Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder Adrenoleukodystrophy, Autosomal, Neonatal Form Adrenoleukodystrophy Neonatal Adrenoleukodystrophy, Neonatal
Legume Allergy	Fabaceae Allergy
Tomato Allergy	Solanum Lycopersicum Fruit Allergy
Neurodegeneration With Brain Iron Accumulation	Nbia Neurodegeneration With Brain Iron Accumulation Disorders Neurodegeneration, With Brain Iron Accumulation
Peanut Allergy	Allergy To Peanuts Peanut Allergic Reaction Allergies Peanut Peanut Hypersensitivity
Byssinosis	Flax-Dressers' Disease Cotton Mill Fever Stripper'S Asthma Monday Morning Fever Cotton Workers' Lung Disease Cotton-Dust Asthma Mill Fever Lung Fibrosis With Byssinosis Airway Disease Due To Cotton Dust Respiratory Tract Disorder Due To Cotton Dust Strippers' Disease Flax-Dressers' Disorder Strippers' Asthma
Chicken Egg Allergy	Gallus Gallus Egg Allergy
Rhizomelic Chondrodysplasia Punctata, Type 2	Rhizomelic Chondrodysplasia Punctata Type 2 Dihydroxyacetonephosphate Acyltransferase Deficiency RCDP2 Dhapat Deficiency Glyceronephosphate O-Acyltransferase Deficiency Gnpat Deficiency Peroxisomal Dihydroxyacetonephosphate Acyltransferase Deficiency Chondrodysplasia Punctata, Rhizomelic, Due To Dihydroxyacetonephosphate Acyltransferase Deficiency Chondrodysplasia Punctata, Rhizomelic, Type 2 Rhizomelic Chondrodysplasia Punctata 2
Latex Allergy
Melon Allergy	Cucumis Melo Fruit Allergy
Spastic Paraplegia 46, Autosomal Recessive	SPG46 Hereditary Spastic Paraplegia 46 Autosomal Recessive Spastic Paraplegia Type 46 Autosomal Recessive Spastic Paraplegia 46 Paraplegia, Spastic, Type 46, Autosomal Recessive
Cholestasis, Benign Recurrent Intrahepatic, 1	Benign Recurrent Intrahepatic Cholestasis BRIC1 Summerskill Syndrome Bric Summerskill-Walshe-Tygstrup Syndrome Cholestasis, Benign Recurrent Intrahepatic Benign Recurrent Intrahepatic Cholestasis 1 Benign Recurrent Intrahepatic Cholestasis Type 1 Bric Type 1 Low Gamma-Gt Familial Intrahepatic Cholestasis Recurrent Familial Intrahepatic Cholestasis Cholestasis, Benign Recurrent Intrahepatic 1 Mild Atp8b1 Deficiency Recurrent Familial Intrahepatic Cholestasis 1 Abcb11-Related Intrahepatic Cholestasis Atp8b1-Related Intrahepatic Cholestasis Cholestasis, Intrahepatic, Recurrent, Benign, Type 1 Progressive Intrahepatic Cholestasis Cholestasis, Progressive Familial Intrahepatic 3 Bric - [Benign Recurrent Intrahepatic Cholestasis]
Cardiomyopathy, Dilated, 1e	Left Ventricular Noncompaction 9 Left Ventricular Noncompaction 5 Dilated Cardiomyopathy 1e Dilated Cardiomyopathy 1s CMD1E Cdcd2 Cardiomyopathy, Dilated, 1y CMD1Y Cardiomyopathy, Dilated, 1s CMD1S Dilated Cardiomyopathy 1y Dilated Cardiomyopathy With Conduction Defect 2 Dilated Cardiomyopathy With Conduction Disorder And Arrhythmia Cardiomyopathy, Dilated, With Conduction Disorder And Arrhythmia Cardiomyopathy, Dilated, With Conduction Defect 2 Cardiomyopathy Dilated With Conduction Defect Type 2 Cardiomyopathy, Dilated 1e Cardiomyopathy, Dilated 1s Cardiomyopathy, Dilated 1y Left Ventricular Non-Compaction 5 LVNC5 Left Ventricular Non-Compaction 9 LVNC9 Cardiomyopathy, Dilated, Type 1e Cardiomyopathy, Dilated, Type 1s Cardiomyopathy, Dilated, Type 1y
Peroxisomal Biogenesis Disorder	Zellweger Spectrum Disorders Peroxisome Biogenesis Disorder-Zellweger Syndrome Spectrum Disorders Of Peroxisome Biogenesis Zellweger Spectrum Zellweger Syndrome Spectrum Peroxisomal Biogenesis Disorders Pbd, Zss Pbd-Zsd Pbd-Zss Pbd-Zellweger Spectrum Disorder Peroxisomal Biogenesis Disorders, Zellweger Syndrome Spectrum Peroxisome Biogenesis Disorder Peroxisome Biogenesis Disorder Spectrum Peroxisome Biogenesis Disorders Zellweger Spectrum Disorder Hyperpipecolic Acidaemia
Peroxisomal Disease	Peroxisomal Disorder Peroxisomal Disorders Peroxisomal Defects
Peroxisome Biogenesis Disorder 1b	Peroxisome Biogenesis Disorder Infantile Refsum Disease Infantile Phytanic Acid Storage Disease PBD1B Refsum Disease, Infantile Adrenoleukodystrophy, Autosomal Neonatal Ird Mild Pbd-Zsd Mild Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder Pbd-Zsd Peroxisome Biogenesis Disorder Spectrum Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder Autosomal Neonatal Adrenoleukodystrophy Refsum Disease Infantile Peroxisome Biogenesis Disorders Peroxisome Biogenesis Disorder, Type 1b
Perrault Syndrome	Gonadal Dysgenesis, Xx Type, With Deafness Ovarian Dysgenesis With Sensorineural Deafness Gonadal Dysgenesis, Xx Type Gonadal Dysgenesis With Auditory Dysfunction, Autosomal Recessive Inheritance Gonadal Dysgenesis With Sensorineural Deafness, Autosomal Recessive Inheritance Xx Gonodal Dysgenesis-Deafness Syndrome Xx Gonodal Dysgenesis-Hearing Loss Syndrome Gonadal Dysgenesis Xx Type Deafness

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (9)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-124824	Methyl gerfelin	Inhibitor	99.9%	Unkown
HY-N15885	12-NBD Stearate	Ligand	/	Unkown
HY-RS03292	CTDSP2 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS12559	SCP2 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS14071	SYCP2 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-180460	SCPI-1		/	Unkown
HY-180500	SCPI-3		/	Unkown
HY-RS20817	Scp2 Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS27331	Scp2 Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Macaca mulatta	SCP2	VGNC	VGNC:100134
Mus musculus	SCP2	MGD	MGI:98254
Canis familiaris	SCP2	VGNC	VGNC:45930
Bos taurus	SCP2	VGNC	VGNC:34361
Rattus norvegicus	SCP2	RGD	RGD:3642
Felis catus	SCP2	VGNC	VGNC:80351
Others	SCP2	NCBI