2. Gene
  3. F13B - coagulation factor XIII B chain Gene

F13B - coagulation factor XIII B 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 B 链

种属: Homo sapiens

同用名: FXIIIB

基因 ID: 2165 | 基因类型: protein coding

关于 F13B

Cytogenetic location: 1q31.3 Genomic coordinates (GRCh38): 1:197,038,741-197,067,260 (from NCBI)

This gene has 3 transcripts (splice variants), 250 orthologues, 39 paralogues and is associated with 2 phenotypes. Restricted expression toward liver (RPKM 37.1).

功能概要

该基因编码凝血因子 XIII B 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。在通过凝血酶裂解激活肽激活后，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。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 coagulation factor XIII B 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 a plasma carrier molecules. Platelet factor XIII is comprised only of 2 A subunits, which are identical to those of plasma origin. Upon activation by the 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. 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]

F13B 基因产物(1)

mRNA Protein Name
NM_001994.3 NP_001985.2 coagulation factor XIII B chain precursor

F13B 蛋白结构

Sushi

Sushi: Sushi repeat (SCR repeat) (47 - 87)

Sushi

Sushi: Sushi repeat (SCR repeat) (91 - 146)

Sushi

Sushi: Sushi repeat (SCR repeat) (153 - 208)

Sushi

Sushi: Sushi repeat (SCR repeat) (213 - 267)

Sushi

Sushi: Sushi repeat (SCR repeat) (274 - 327)

Sushi

Sushi: Sushi repeat (SCR repeat) (336 - 389)

Sushi

Sushi: Sushi repeat (SCR repeat) (396 - 450)

Sushi

Sushi: Sushi repeat (SCR repeat) (524 - 578)

  • 0
  • 200
  • 400
  • 600
  • 661 a.a.
蛋白主名 其他名称

coagulation factor XIII B chain

TGase

重组 F13B 蛋白

目录号 产品名 蛋白编号 纯度
HY-P74238 Coagulation factor XIII B/F13B Protein, Human (HEK293, His) P05160 (E21-T661) ≥95%

关联疾病

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

Factor Xiiib Deficiency

Factor Xiii Subunit B Deficiency

FA13BD

F13 Deficiency Type 1

Type I F13 Deficiency
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
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
Thrombosis

Thrombosis Of Blood Vessel
Retinitis Pigmentosa 12

RP12

Retinitis Pigmentosa With Or Without Paraarteriolar Preservation Of Retinal Pigment Epithelium

Rp With Or Without Preserved Paraarteriole Retinal Pigment Epithelium

Rp With Or Without Pprpe

Retinitis Pigmentosa-12
Hemarthrosis

Haemarthrosis Of Shoulder Joint

Haemarthrosis Of The Ankle And Foot

Haemarthrosis Of The Pelvic Region And Thigh

Hemarthrosis Involving Ankle And Foot

Hemarthrosis Involving Forearm

Hemarthrosis Involving Hand

Hemarthrosis Involving Lower Leg

Hemarthrosis Involving Pelvic Region And Thigh

Hemarthrosis Involving Shoulder Region

Hemarthrosis Involving Upper Arm

Hemarthrosis Of Ankle And/Or Foot

Hemarthrosis Of Forearm

Hemarthrosis Of Hand

Hemarthrosis Of Lower Leg

Hemarthrosis Of Shoulder

Hemarthrosis Of Shoulder Region

Hemarthrosis Of The Ankle And Foot

Hemarthrosis Of The Ankle And/Or Foot

Hemarthrosis Of The Forearm

Hemarthrosis Of The Hand

Hemarthrosis Of The Lower Leg

Hemarthrosis Of The Pelvic Region And Thigh

Hemarthrosis Of The Shoulder Region

Hemarthrosis Of The Upper Arm

Hemarthrosis Of Upper Arm
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-RS04629 F13B Human Pre-designed siRNA Set A Pre-designed Sets / 否

直系同源

种属 基因名 来源 基因 ID
Mus musculus F13B MGD MGI:88379
Macaca mulatta F13B VGNC VGNC:72463
Felis catus F13B VGNC VGNC:62022
Bos taurus F13B VGNC VGNC:28681
Canis familiaris F13B VGNC VGNC:40546
Rattus norvegicus F13B RGD RGD:1311668
Macaca fascicularis F13B NCBI
Others F13B NCBI
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