Human Ferritin Heavy Chain Ready-To-Use IHC Kit (免疫组化试剂盒) - IHC-P | Bioss
货号：IHC0430H
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概述
产品编号
IHC0430H
英文名称
Human Ferritin Heavy Chain Ready-To-Use IHC Kit
中文名称
人铁蛋白即用型免疫组化试剂盒
英文别名
Apoferritin; Cell proliferation inducing gene 15 protein; F HC; Ferritin H subunit; Ferritin heavy chain; Ferritin heavy polypeptide 1; FHC; FRIH; FTH 1; FTH; FTH1; FTH1 protein; FTHL 6; FTHL6; Iron overload autosomal dominant; MGC104426; OK/SW-cl.84; PIG 15; PIG15; Placenta immunoregulatory factor; PLIF; Proliferation inducing gene 15 protein; Proliferation inducing protein 15; FRIH_HUMAN; HFE5; HFt; MFH
描述
IHC0430H is a ready-to-use IHC kit for staining of human Ferritin Heavy Chain. The kit provides all reagents, from antigen retrieval to cover slip mounting, that require little to no diluting or handling prior to use. Simply apply the reagents to your sample slide according to the protocol and you're steps away from obtaining high-quality IHC data.
适用样品基质
FFPE tissue
应用范围
Immunohistochemistry
保存条件
Please store components at the temperatures indicated on the individual tube labels. The kit is stable for 6 months from the date of receipt.
背景资料
FTH1 is the heavy subunit of ferritin, the major intracellular iron storage protein in prokaryotes and eukaryotes. It is composed of 24 subunits of the heavy and light ferritin chains. Variation in ferritin subunit composition may affect the rates of iron uptake and release in different tissues. A major function of ferritin is the storage of iron in a soluble and nontoxic state. Defects in ferritin proteins are associated with several neurodegenerative diseases. This gene has multiple pseudogenes. Several alternatively spliced transcript variants have been observed, but their biological validity has not been determined.
产品应用
产品应用: IHC-P
交叉反应
交叉反应: Human
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靶标
基因名
FTH1
蛋白名
Ferritin heavy chain
亚基
Oligomer of 24 subunits (PubMed:9159481).
There are two types of subunits: L (light) chain and H (heavy) chain (PubMed:9159481).
The major chain can be light or heavy, depending on the species and tissue type. In the human liver, the heavy chain is predominant (PubMed:9159481).
The functional molecule forms a roughly spherical shell with a diameter of 12 nm and contains a central cavity into which the insoluble mineral iron core is deposited (PubMed:9159481).
Interacts with NCOA4; NCOA4 promotes targeting of the iron-binding ferritin complex to autolysosomes following starvation or iron depletion (PubMed:24695223).
亚细胞定位
#Cytoplasm
#Cytoplasmic vesicle
#Lysosome
组织特异性
Expressed in the liver.
疾病
Hemochromatosis 5 (HFE5)
Note
The disease is caused by variants affecting the gene represented in this entry. In a Japanese family affected by HFE5, a single point mutation has been detected in the iron-responsive element (IRE) in the 5'-UTR of FTH1 mRNA. This mutation leads to an increased binding affinity for iron regulatory protein and thereby to the efficient suppression of mRNA translation
Description
A disorder of iron metabolism characterized by iron overload. Excess iron is deposited in a variety of organs leading to their failure, and resulting in serious illnesses including cirrhosis, hepatomas, diabetes, cardiomyopathy, arthritis, and hypogonadotropic hypogonadism. Severe effects of the disease usually do not appear until after decades of progressive iron loading.
Neurodegeneration with brain iron accumulation 9 (NBIA9)
Note
The disease is caused by variants affecting the gene represented in this entry
Description
An autosomal dominant neurodegenerative disorder associated with iron accumulation, primarily in the basal ganglia. It is characterized by global developmental delay apparent from infancy, and progressive decline of motor and cognitive skills. Clinical features include delayed walking or inability to walk, spasticity with hyperreflexia, ataxia, dystonia, and poor or absent language.
相似性
Belongs to the ferritin family.
功能
Stores iron in a soluble, non-toxic, readily available form. Important for iron homeostasis. Has ferroxidase activity (PubMed:9003196).
Iron is taken up in the ferrous form and deposited as ferric hydroxides after oxidation (PubMed:9003196).
Also plays a role in delivery of iron to cells (By similarity).
Mediates iron uptake in capsule cells of the developing kidney (By similarity).
Delivery to lysosomes is mediated by the cargo receptor NCOA4 for autophagic degradation and release of iron (PubMed:24695223, PubMed:26436293).