2. Gene
  3. F13A1 - coagulation factor XIII A chain Gene

F13A1 - coagulation factor XIII A chain Gene

  • 基因
  • 蛋白
  • 疾病
  • 相关产品
  • 直系同源

中文名称：凝血因子 XIII A 链

种属: Homo sapiens

同用名: F13A

基因 ID: 2162 | 基因类型: protein coding

关于 F13A1

Cytogenetic location: 6p25.1 Genomic coordinates (GRCh38): 6:6,144,084-6,320,662 (from NCBI)

This gene has 6 transcripts (splice variants), 291 orthologues, 8 paralogues and is associated with 4 phenotypes. Biased expression in placenta (RPKM 246.8), urinary bladder (RPKM 65.1) and 12 other tissues.

功能概要

该基因编码凝血因子 XIII A 亚基。凝血因子 XIII 是凝血级联反应中最后激活的酶原。血浆因子 XIII 是由 2 个 A 亚基和 2 个 B 亚基组成的异四聚体。 A 亚基具有催化功能，B 亚基不具有酶活性，可作为血浆载体分子。血小板 XIII 因子仅由 2 个 A 亚基组成，与血浆来源的亚基相同。激活肽被凝血酶裂解后，在钙离子存在的情况下，血浆因子 XIII 解离其 B 亚基并产生与血小板因子 XIII 相同的活性酶，即因子 XIIIa。这种酶作为转谷氨酰胺酶催化纤维蛋白分子之间形成γ-谷氨酰-ε-赖氨酸交联，从而稳定纤维蛋白凝块。它还将 alpha-2-纤溶酶抑制剂或纤连蛋白与纤维蛋白的 alpha 链交联。因子 XIII 缺乏症分为两类： I 型缺乏症，其特征是同时缺乏 A 和 B 亚基；和 II 型缺陷，其特征是仅缺乏 A 亚基。这些缺陷会导致终生出血倾向、伤口愈合不良和习惯性流产。[RefSeq 提供，2008 年 7 月]

This gene encodes the coagulation factor XIII A subunit. Coagulation factor XIII is the last zymogen to become activated in the blood coagulation cascade. Plasma factor XIII is a heterotetramer composed of 2 A subunits and 2 B subunits. The A subunits have catalytic function, and the B subunits do not have enzymatic activity and may serve as plasma carrier molecules. Platelet factor XIII is comprised only of 2 A subunits, which are identical to those of plasma origin. Upon cleavage of the activation peptide by Thrombin and in the presence of calcium ion, the plasma factor XIII dissociates its B subunits and yields the same active Enzyme, factor XIIIa, as platelet factor XIII. This Enzyme acts as a transglutaminase to catalyze the formation of gamma-glutamyl-epsilon-lysine crosslinking between fibrin molecules, thus stabilizing the fibrin clot. It also crosslinks alpha-2-plasmin inhibitor, or fibronectin, to the alpha chains of fibrin. Factor XIII deficiency is classified into two categories: type I deficiency, characterized by the lack of both the A and B subunits; and type II deficiency, characterized by the lack of the A subunit alone. These defects can result in a lifelong bleeding tendency, defective wound healing, and habitual abortion. [provided by RefSeq, Jul 2008]

F13A1 基因产物(1)

mRNA Protein Name
NM_000129.4 NP_000120.2 coagulation factor XIII A chain

F13A1 蛋白结构

Transglut_N

Transglut_N: Transglutaminase family (46 - 166)

Transglut_core

Transglut_core: Transglutaminase-like superfamily (311 - 397)

Transglut_C

Transglut_C: Transglutaminase family, C-terminal ig like domain (519 - 623)

Transglut_C

Transglut_C: Transglutaminase family, C-terminal ig like domain (631 - 727)

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  • 732 a.a.
蛋白主名 其他名称

coagulation factor XIII A chain

FSF, A subunit

重组 F13A1 蛋白

目录号 产品名 蛋白编号 纯度
HY-P70226 Coagulation factor XIII A/F13A Protein, Human (HEK293, His) AAH27963.1 (G39-M732) ≥95%

关联疾病

疾病名称 别名
Factor Xiii, A Subunit, Deficiency Of

Factor Xiiia Deficiency

Factor Xiii Subunit A Deficiency

FA13AD

F13 Deficiency Type 2

Type Ii F13 Deficiency
Thrombophilia Due To Thrombin Defect

Venous Thromboembolism

Venous Thrombosis

Thrombophilia Due To Factor 2 Defect

Thromboembolism

THPH1

Thromboembolism, Susceptibility To

Venous Thromboembolism, Susceptibility To

Venous Thrombosis, Protection Against

Prothrombin-Related Thrombophilia

Hyperprothrombinemia

Venous Thrombosis, Susceptibility To

Thrombophilia 1 Due To Thrombin Defect

F2-Related Thrombophilia

Factor Ii-Related Thrombophilia

Prothrombin 20210g>A Thrombophilia

Prothrombin G20210a Thrombophilia

Prothrombin Thrombophilia
Factor Xiii Deficiency

Hereditary Factor Xiii Deficiency Disease

Deficiency, Laki-Lorand Factor

Congenital Factor Xiii Deficiency

Fibrin Stabilizing Factor Deficiency

Deficiency, Factor Xiii

Factor Xiii Deficiency Disease

Deficiency Of Factor Xiii

Fibrin-Stabilizing Factor Deficiency

Factor Xiii Deficiency, Congenital
Myocardial Infarction

Heart Attack

Myocardial Infarction, Susceptibility To

Myocardial Infarction 1

Myocardial Infarction, Protection Against

Myocardial Infarction, Decreased Susceptibility To

Myocardial Infarction, Decreased

Myocardial Infarct

MCI1

Premature Myocardial Infarction

Myocardial Infarction, Susceptibility To, Type 1
Cutaneous Fibrous Histiocytoma

Dermatofibroma

Fibrohistiocytic Tumor

Benign Cutaneous Fibrous Histiocytoma

Fibrous Histiocytoma Of Skin

Fibrous Xanthoma Of Skin

Pleomorphic Fibroma

Sclerosing Angioma

Sclerosing Angioma Of Skin

Fibrous Histiocytoma
Granuloma Annulare

Granulome Annulare

Ga - [Granuloma Annulare]
Fibrous Histiocytoma

Benign Fibrous Histiocytoma

Histiocytoma, Benign Fibrous

Fibroxanthoma

Histiocytoma Fibrous
Xanthoma Disseminatum

Montgomery Syndrome
Dermatofibrosarcoma Protuberans

DFSP

Dermatofibrosarcoma

Giant Cell Fibroblastoma

Metastatic Dermatofibrosarcoma Protuberans

Familial Dermatofibrosarcoma Protuberans

Darier-Ferrand Tumor

Darier-Hoffmann Tumor
Juvenile Xanthogranuloma

Multiple Eruptive Juvenile Xanthogranuloma

Naevoxanthoendothelioma

Xanthoma Neviforme

Xanthogranuloma, Juvenile
Thrombosis

Thrombosis Of Blood Vessel
Fibroma
Angiolipoma
Pulmonary Embolism

Pulmonary Artery Embolism

Pulmonary Embolus

Pulmonary Emboli
Neurofibroma

Neurofibromas

Neurofibromatoses

Nerve Sheath Tumors
Priapism

Mentulagra

Priapism, Familial Idiopathic

Familial Idiopathic Priapism

Pathologic Erection

Painful Erection
Malignant Fibrous Histiocytoma

Undifferentiated Pleomorphic Sarcoma

Ups

Histiocytoma, Malignant Fibrous

Fibroxanthosarcoma

Mfh

Malignant Fibrohistiocytic Tumors

Histiocytoma, Fibrous, Malignant
Langerhans Cell Histiocytosis

Histiocytosis X

Lch

Langerhans Cell Granulomatosis

Langerhans-Cell Histiocytosis

Letterer-Siwe Disease

Hashimoto-Pritzger Disease

Histiocytosis, Langerhans-Cell

Langerhan'S Cell Histiocytosis

Letterer-Siwe Disease Involving Intra-Abdominal Lymph Nodes

Letterer-Siwe Disease Involving Intrapelvic Lymph Nodes

Letterer-Siwe Disease Involving Intrathoracic Lymph Nodes

Letterer-Siwe Disease Involving Lymph Nodes Of Axilla And Upper Limb

Letterer-Siwe Disease Involving Lymph Nodes Of Head, Face And Neck

Letterer-Siwe Disease Involving Lymph Nodes Of Head, Face, And Neck

Letterer-Siwe Disease Involving Lymph Nodes Of Inguinal Region And Lower Limb

Letterer-Siwe Disease Involving Lymph Nodes Of Multiple Sites

Letterer-Siwe Disease Involving Spleen

Letterer-Siwe Disease Of Intra-Abdominal Lymph Nodes

Letterer-Siwe Disease Of Intrapelvic Lymph Nodes

Letterer-Siwe Disease Of Intrathoracic Lymph Nodes

Letterer-Siwe Disease Of Lymph Nodes Of Axilla And Upper Limb

Letterer-Siwe Disease Of Lymph Nodes Of Axilla And/Or Upper Limb

Letterer-Siwe Disease Of Lymph Nodes Of Head, Face And Neck

Letterer-Siwe Disease Of Lymph Nodes Of Head, Face And/Or Neck

Letterer-Siwe Disease Of Lymph Nodes Of Inguinal Region Amd/Or Lower Limb

Letterer-Siwe Disease Of Lymph Nodes Of Inguinal Region And Lower Limb

Letterer-Siwe Disease Of Lymph Nodes Of Inguinal Region And/Or Lower Limb

Letterer-Siwe Disease Of Lymph Nodes Of Multiple Sites

Letterer-Siwe Disease Of Spleen

Familial Letterer-Siwe Disease

Langerhans-Cell Histiocytosis Nos
Thrombophilia

Hypercoagulability State
Dysfibrinogenemia, Congenital

Dysfibrinogenemia

Hypodysfibrinogenemia

Familial Dysfibrinogenemia

Familial Hypodysfibrinogenemia

Hypodysfibrinogenemia, Congenital

Dysfibrinogenemia, Familial

Congenital Dysfibrinogenemia

DYSFIBRIN
Hemangiopericytoma, Malignant

Hemangiopericytoma

Haemangiopericytic Meningioma

Malignant Hemangiopericytoma

Solitary Fibrous Tumor
Cerebrovascular Disease

Cerebrovascular Disorder

Cerebrovascular Accident

Cerebrovascular Disorders

Cva

Stroke
Hemorrhagic Disease

Hemorrhagic Disorders

Hemorrhagic Diathesis

Hemorrhagic Diseases

Bleeding Tendency

Acquired Coagulation Factor Inhibitor Disorder

Acquired Inhibitor Of Coagulation

Antithrombinaemia

Antithromboplastinogenaemia

Circulating Anticoagulant Disorder

Haemorrhagic Disorder Due To Antithrombinaemia

Haemorrhagic Disorder Due To Excess Administered Heparin

Antithromboplastinaemia

Haemorrhagic Disorder Due To Hyperheparinemia
Dedifferentiated Liposarcoma

Ddls

Liposarcoma Dedifferentiated

Liposarcoma, Dedifferentiated
Castleman Disease

Angiofollicular Ganglionic Hyperplasia

Angiofollicular Lymph Hyperplasia

Giant Lymph Node Hyperplasia

Castleman'S Disease

Angiofollicular Lymph Node Hyperplasia

Lymphoid Hamartoma

Benign Giant Lymphoma

Angiolymphoid Hyperplasia
Localized Scleroderma

Morphea

Localised Scleroderma

Localized Fibrosing Scleroderma

Circumscribed Scleroderma

Localised Morphea

Localised Morphoea

Localized Morphea

Scleroderma, Circumscribed Or Localised

Scleroderma, Circumscribed Or Localized

Scleroderma, Localized
Pancytopenia
Basal Cell Carcinoma

Basal Cell Cancer

Basal Cell Neoplasm

Basal Cell Carcinoma Of Skin

Malignant Basal Cell Tumor

Basal Cell Tumor

Epithelioma Basal Cell

Malignant Basal Cell Neoplasm

Rodent Ulcer

Carcinoma Basal Cell

Neoplasms, Basal Cell

Basal Cell Carcinomas

Experimental Organism Basal Cell Carcinoma

Nodulo-Ulcerative Basal Cell Carcinoma

Basalioma

Basal Cell Epithelioma Of Skin

Bcc - [Basal Cell Carcinoma] Of Skin

Rodent Ulcer Of Skin

Rodent Ulcer Of Unspecified Site

Basal Cell Epithelioma Of Unspecified Site
Skin Disease

Skin Diseases

Genodermatosis

Abnormality Of The Skin

Skin Diseases, Genetic

Skin And Subcutaneous Tissue Disease

Dermatologic Disorders
疾病名称 别名
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

相关产品

Pre-clinical Phase
生物活性分子 (1)
Cat. No. 产品名 作用方式
纯度 是否罕见病
HY-RS04628 F13A1 Human Pre-designed siRNA Set A Pre-designed Sets / 否

直系同源

种属 基因名 来源 基因 ID
Bos taurus F13A1 VGNC VGNC:28680
Mus musculus F13A1 MGD MGI:1921395
Canis familiaris F13A1 VGNC VGNC:40545
Macaca mulatta F13A1 VGNC VGNC:72462
Rattus norvegicus F13A1 RGD RGD:621495
Felis catus F13A1 VGNC VGNC:62021
Others F13A1 NCBI
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