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FLASH GENE

Symbol

PROS1

contributors: mct - updated : 22-12-2009

HGNC name	protein S (alpha)
HGNC id	9456

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

Corresponding disease	PROS1 pro-S deficiency
Location	3q11.2      Physical location : 93.591.881 - 93.692.934
Synonym name	vitamin K-dependent plasma protein S
	protein Sa
Synonym symbol(s)	PROS, PS21, PS22, PS23, PS24, PS25, PSA

DNA

TYPE	functioning gene
STRUCTURE	101.05 kb     15 Exon(s)

10 Kb 5' upstream gene genomic sequence study

regulatory sequence	Promoter
	Binding site   hormone   HRE
text structure	the liver-specific factors hepatocyte nuclear factor 1 (HNF1) and HNF4 bind regions of the protein S promoter, which lie within the identified protein binding sites V and VIII, respectively, and HNF4 activates the promoter
	PROS1 promoter is responsive to progesterone and other progestins via a mechanism involving PR-B (progesterone receptor isoform B) interacting with a PRE (progesterone response element) (Hughes 2007)

MAPPING

cloned

Y

linked

N

status

confirmed

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_000313 15 - 3595 NP_000304 - 676 - 2008 18680534

EXPRESSION

Type	ubiquitous

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol Digestive liver       highly Nervous brain       highly Reproductive female system placenta     highly Skin/Tegument skin       highly

tissue

System Tissue Tissue level 1 Tissue level 2 Level Pubmed Species Stage Rna symbol Blood / hematopoietic plasma

cells

System Cell Pubmed Species Stage Rna symbol Blood/Hematopoietic platelet

cell lineage

cell lines

fluid/secretion

at STAGE

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	one gamma carboxyglutamic domain
	two laminin A/neurexin/sex hormone-binding globulin repeats (LNS)
	four EGF-like domains (GLA domain)

conjugated

GlycoP

mono polymer

homomer , monomer , polymer

HOMOLOGY

Homologene

FAMILY

AXL family of receptor tyrosine kinases

CATEGORY

regulatory

SUBCELLULAR LOCALIZATION	extracellular
	intracellular
	intracellular,cytoplasm,cytosolic,granule
text	secreted from platelets alpha granules

basic FUNCTION	co-factor for activated protein C to inactivate coagulation factors
	playing a pivotal role in inflammation
	with TFPI, are major regulators of thrombin generation both in the absence and presence of activated protein C (APC)
	with TFPI, act together in down-regulating coagulation )

CELLULAR PROCESS

PHYSIOLOGICAL PROCESS

coagulation/hemostasis , inflammation

PATHWAY

metabolism

signaling

a component

INTERACTION

DNA

RNA

small molecule	metal binding,
	Zn(2+) is required for PROS1-direct and efficient FXa binding and may play a role in stabilizing PROS1 conformation

protein	binding factors Xa,Va to inhibit prothrombinase
	interfering with plasminogen binding on cell surface
	cofactor for activated protein C (APC), which inactivates coagulation factors (F) Va and VIIIa
	dependence on vitamin K-dependent PROS1 for eukaryotic cell secretion of C4BPB

cell & other

REGULATION

inhibited by

binding to C4BP

Other

vitamin K-dependent

ASSOCIATED DISORDERS

corresponding disease(s)

PROS1

related resource

Protein S Deficiency: A database of Mutations

Susceptibility

Variant & Polymorphism

Candidate gene

Marker

Therapy target

ANIMAL & CELL MODELS