PHYH and F8

Number of citations: 0 (PubMedID 11574539)
Data Source: HPRD (two hybrid, in vitro, in vivo)

		PHYH	F8
Name		phytanoyl-CoA 2-hydroxylase	coagulation factor VIII
Structure
GO Annotations	Cellular Component	Peroxisome Peroxisomal Matrix Cytosol 9+0 Non-motile Cilium	Extracellular Region Extracellular Space Endoplasmic Reticulum Lumen Golgi Lumen Plasma Membrane COPII-coated ER To Golgi Transport Vesicle Platelet Alpha Granule Lumen Endoplasmic Reticulum-Golgi Intermediate Compartment Membrane
	Molecular Function	Catalytic Activity Protein Binding Ferrous Iron Binding Oxidoreductase Activity Carboxylic Acid Binding L-ascorbic Acid Binding Metal Ion Binding Phytanoyl-CoA Dioxygenase Activity Dioxygenase Activity	Copper Ion Binding Protein Binding Oxidoreductase Activity Metal Ion Binding
	Biological Process	Fatty Acid Alpha-oxidation 2-oxoglutarate Metabolic Process Fatty Acid Metabolic Process Isoprenoid Metabolic Process 2-oxobutyrate Catabolic Process Methyl-branched Fatty Acid Metabolic Process	Acute-phase Response Blood Coagulation Blood Coagulation, Intrinsic Pathway Hemostasis
Pathways		Alpha-oxidation of phytanate Peroxisomal protein import Peroxisomal protein import TYSND1 cleaves peroxisomal proteins	Platelet degranulation Intrinsic Pathway of Fibrin Clot Formation Common Pathway of Fibrin Clot Formation Gamma carboxylation, hypusinylation, hydroxylation, and arylsulfatase activation COPII-mediated vesicle transport Cargo concentration in the ER Defective factor IX causes thrombophilia Defective F8 accelerates dissociation of the A2 domain Defective F8 cleavage by thrombin Defective F8 binding to von Willebrand factor Defective F8 binding to the cell membrane Defective cofactor function of FVIIIa variant Defective F8 secretion Defective F9 variant does not activate FX Defective F8 sulfation at Y1699
Drugs		Antihemophilic factor, human recombinant Ascorbic acid Lonoctocog alfa Moroctocog alfa	Drotrecogin alfa Coagulation Factor IX (Recombinant) TB-402 Thrombin Protein C Human thrombin Thrombin alfa Vonicog alfa Von Willebrand factor human Anti-inhibitor coagulant complex Coagulation Factor IX Human Nonacog beta pegol Damoctocog alfa pegol Rurioctocog alfa pegol
Diseases		Refsum disease; Heredopathia atactica polyneuritiformis	Hemophilia; Hemophilia A (HEMA); Hemophilia B (HEMB); von Willebrand disease (VWD)
GWAS		No GWAS studies recorded	Antineutrophil cytoplasmic antibody-associated vasculitis ( 22808956 ) Factor VIII levels ( 30586737 ) Red blood cell traits ( 23446634 ) Thrombosis ( 26908601 ) Venous thromboembolism ( 31420334 )
Interacting Genes		11 interacting genes: ACSL6 APP F8 FAM9B FKBP4 MAGEA11 PATJ PEX7 PHYHIP SIRPA TOM1L1	17 interacting genes: ASGR2 CALR CANX F10 F2 F9 GGA1 HSPA5 LMAN1 LRP1 MCFD2 PHYH PROC PROS1 TSC22D2 UBQLN1 VWF
Entrez ID		5264	2157
HPRD ID		03609	02384
Ensembl ID		ENSG00000107537	ENSG00000185010
Uniprot IDs		O14832	P00451
PDB IDs		2A1X	1CFG 1D7P 1FAC 1IQD 2R7E 3CDZ 3HNB 3HNY 3HOB 3J2Q 3J2S 4BDV 4KI5 4PT6 4XZU 5K8D 6MF0 6MF2 7K66 7KBT 7KWO 8G6I 8TY1 9D5D
GO Terms Enriched among Interactors		None	Coagulation Blood Coagulation Hemostasis Calcium Ion Binding Regulation Of Body Fluid Levels Endoplasmic Reticulum Lumen Golgi Lumen Protein Folding In Endoplasmic Reticulum Endoplasmic Reticulum Quality Control Compartment ERAD Pathway Unfolded Protein Binding Serine-type Peptidase Activity Proteolysis Negative Regulation Of Blood Coagulation Serine-type Endopeptidase Activity Negative Regulation Of Hemostasis Negative Regulation Of Coagulation Carbohydrate Binding Protein Folding Negative Regulation Of Wound Healing Regulation Of Hemostasis Regulation Of Blood Coagulation Protein Transport Endoplasmic Reticulum Endoplasmic Reticulum-Golgi Intermediate Compartment Membrane Endoplasmic Reticulum-Golgi Intermediate Compartment Response To Endoplasmic Reticulum Stress Endopeptidase Activity Protein-folding Chaperone Binding Fibrinolysis Establishment Of Protein Localization D-mannose Binding Protein Catabolic Process Regulation Of Wound Healing External Side Of Plasma Membrane Lumenal Side Of Endoplasmic Reticulum Membrane Extracellular Space Endoplasmic Reticulum Membrane Regulation Of Intracellular Signal Transduction Regulation Of Biological Quality Extracellular Exosome Regulation Of Response To Wounding Extracellular Matrix Vesicle-mediated Transport Peptidase Activity Regulation Of Protein Folding In Endoplasmic Reticulum Positive Regulation Of Transcytosis Alpha-2 Macroglobulin Receptor Activity Apolipoprotein Receptor Activity 2-oxobutyrate Catabolic Process