製品: HFE2 Antibody
カタログ: DF7290
タンパク質の説明: Rabbit polyclonal antibody to HFE2
アプリケーション: WB IHC
反応性: Human, Mouse, Rat
分子量: 45kDa(Observed); 45kD(Calculated).
ユニプロット: Q6ZVN8
RRID: AB_2839229

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製品説明

ソース:
Rabbit
アプリケーション:
WB 1:500-1:2000, IHC 1:50-1:200
*The optimal dilutions should be determined by the end user. For optimal experimental results, antibody reuse is not recommended.
*Tips:

WB: For western blot detection of denatured protein samples. IHC: For immunohistochemical detection of paraffin sections (IHC-p) or frozen sections (IHC-f) of tissue samples. IF/ICC: For immunofluorescence detection of cell samples. ELISA(peptide): For ELISA detection of antigenic peptide.

反応性:
Human,Mouse,Rat
クローナリティ:
Polyclonal
特異性:
HFE2 Antibody detects endogenous levels of total HFE2.
RRID:
AB_2839229
引用形式: Affinity Biosciences Cat# DF7290, RRID:AB_2839229.
コンジュゲート:
Unconjugated.
精製:
The antiserum was purified by peptide affinity chromatography using SulfoLink™ Coupling Resin.
保存:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. Store at -20 °C. Stable for 12 months from date of receipt.
別名:

折りたたみ/展開

2310035L15Rik; 5230400G09Rik; AI414844; AI789733; DL M; Haemojuvelin; HEMOCHROMATOSIS; HEMOCHROMATOSIS DUE TO DEFECT IN HEMOJUVELIN; HEMOCHROMATOSIS DUE TO DEFECT IN HEPCIDIN ANTIMICROBIAL PEPTIDE; HEMOCHROMATOSIS JUVENILE; Hemochromatosis type 2 (juvenile); Hemochromatosis type 2; Hemochromatosis type 2 protein; Hemochromatosis type 2 protein homolog; HEMOCHROMATOSIS, TYPE 2A; HEMOCHROMATOSIS, TYPE 2B; Hemojuvelin; HFE 2; Hfe2; HFE2A; HJV; JH; Juvenile; MGC23953; OTTHUMP00000059680; Repulsive guidance molecule c; RGM C; RGM domain family member C; RGMC;

免疫原

免疫原:

A synthesized peptide derived from human HFE2, corresponding to a region within N-terminal amino acids.

Uniprot:
遺伝子(ID):
発現特異性:
Q6ZVN8 RGMC_HUMAN:

Adult and fetal liver, heart, and skeletal muscle.

タンパク質の説明:
The product of this gene is involved in iron metabolism. It may be a component of the signaling pathway which activates hepcidin or it may act as a modulator of hepcidin expression. It could also represent the cellular receptor for hepcidin. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. Defects in this gene are the cause of hemochromatosis type 2A, also called juvenile hemochromatosis (JH). JH is an early-onset autosomal recessive disorder due to severe iron overload resulting in hypogonadotrophic hypogonadism, hepatic fibrosis or cirrhosis and cardiomyopathy, occurring typically before age of 30.
タンパク質配列:
MGEPGQSPSPRSSHGSPPTLSTLTLLLLLCGHAHSQCKILRCNAEYVSSTLSLRGGGSSGALRGGGGGGRGGGVGSGGLCRALRSYALCTRRTARTCRGDLAFHSAVHGIEDLMIQHNCSRQGPTAPPPPRGPALPGAGSGLPAPDPCDYEGRFSRLHGRPPGFLHCASFGDPHVRSFHHHFHTCRVQGAWPLLDNDFLFVQATSSPMALGANATATRKLTIIFKNMQECIDQKVYQAEVDNLPVAFEDGSINGGDRPGGSSLSIQTANPGNHVEIQAAYIGTTIIIRQTAGQLSFSIKVAEDVAMAFSAEQDLQLCVGGCPPSQRLSRSERNRRGAITIDTARRLCKEGLPVEDAYFHSCVFDVLISGDPNFTVAAQAALEDARAFLPDLEKLHLFPSDAGVPLSSATLLAPLLSGLFVLWLCIQ

研究背景

機能:

Acts as a bone morphogenetic protein (BMP) coreceptor. Through enhancement of BMP signaling regulates hepcidin (HAMP) expression and regulates iron homeostasis.

PTMs:

Autocatalytically cleaved at low pH; the two chains remain linked via two disulfide bonds. Also proteolytically processed by TMPRSS6, several fragments being released in the extracellular space; regulates HJV activity in BMP signaling and thefore iron homeostasis.

細胞の位置付け:

Cell membrane>Lipid-anchor.
Note: Also released in the extracellular space.

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionSubcellular location
組織特異性:

Adult and fetal liver, heart, and skeletal muscle.

タンパク質ファミリー:

Belongs to the repulsive guidance molecule (RGM) family.

Restrictive clause

 

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