HBB Polyclonal Antibody | MyBioSource MBS127849 product information

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product summary

company name :

MyBioSource

product type :

antibody

product name :

HBB Polyclonal Antibody

catalog :

MBS127849

quantity :

0.05 mL

price :

285 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse

application :

western blot, immunohistochemistry, immunoprecipitation

product information

catalog number :

MBS127849

products type :

Antibody

products full name :

HBB Polyclonal Antibody

products short name :

HBB

products name syn :

HBB; Hemoglobin subunit beta; Beta-globin; Hemoglobin beta chain; LVV-hemorphin-7; CD113t-C; beta-globin

other names :

Hemoglobin subunit beta; Hemoglobin subunit beta; hemoglobin subunit beta; beta globin chain; hemoglobin beta chain; hemoglobin, beta; Beta-globin; Hemoglobin beta chainCleaved into the following 2 chains:LVV-hemorphin-7; Spinorphin

products gene name :

HBB

other gene names :

HBB; HBB; CD113t-C; beta-globin

uniprot entry name :

HBB_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Human, Mouse

sequence length :

147

purity :

Affinity Purification

storage stability :

Store at -20 degree C. Avoid freeze / thaw cycles.

tested application :

Western Blot (WB), Immunohistochemistry (IHC), Immunoprecipitation (IP)

app notes :

WB: 1:500 - 1:2000. IHC: 1:50 - 1:100. IP: 1:20-1:50

other info1 :

Species: Human

other info2 :

Immunogen: Recombinant protein of human HBB. Calculated Molecular Weight: 16kDa. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

products categories :

Polyclonal

products description :

The alpha (HBA) and beta (HBB) loci determine the structure of the 2 types of polypeptide chains in adult hemoglobin, Hb A. The normal adult hemoglobin tetramer consists of two alpha chains and two beta chains. Mutant beta globin causes sickle cell anemia. Absence of beta chain causes beta-zero-thalassemia. Reduced amounts of detectable beta globin causes beta-plus-thalassemia. The order of the genes in the beta-globin cluster is 5'-epsilon -- gamma-G -- gamma-A -- delta -- beta--3'. [provided by RefSeq, Jul 2008]

ncbi gi num :

56749856

ncbi acc num :

P68871.2

uniprot acc num :

P68871

ncbi mol weight :

16kDa

ncbi pathways :

African Trypanosomiasis Pathway (194384); African Trypanosomiasis Pathway (194323); Binding And Uptake Of Ligands By Scavenger Receptors Pathway (771599); Erythrocytes Take Up Carbon Dioxide And Release Oxygen Pathway (645347); Erythrocytes Take Up Oxygen And Release Carbon Dioxide Pathway (645348); Factors Involved In Megakaryocyte Development And Platelet Production Pathway (187196); Folate Metabolism Pathway (198833); Hemostasis Pathway (106028); Malaria Pathway (152665); Malaria Pathway (152657)

ncbi summary :

uniprot summary :

HBB: Involved in oxygen transport from the lung to the various peripheral tissues. Defects in HBB may be a cause of Heinz body anemias (HEIBAN). This is a form of non-spherocytic hemolytic anemia of Dacie type 1. After splenectomy, which has little benefit, basophilic inclusions called Heinz bodies are demonstrable in the erythrocytes. Before splenectomy, diffuse or punctate basophilia may be evident. Most of these cases are probably instances of hemoglobinopathy. The hemoglobin demonstrates heat lability. Heinz bodies are observed also with the Ivemark syndrome (asplenia with cardiovascular anomalies) and with glutathione peroxidase deficiency. Defects in HBB are the cause of beta-thalassemia (B-THAL). A form of thalassemia. Thalassemias are common monogenic diseases occurring mostly in Mediterranean and Southeast Asian populations. The hallmark of beta-thalassemia is an imbalance in globin-chain production in the adult HbA molecule. Absence of beta chain causes beta(0)-thalassemia, while reduced amounts of detectable beta globin causes beta(+)-thalassemia. In the severe forms of beta-thalassemia, the excess alpha globin chains accumulate in the developing erythroid precursors in the marrow. Their deposition leads to a vast increase in erythroid apoptosis that in turn causes ineffective erythropoiesis and severe microcytic hypochromic anemia. Clinically, beta-thalassemia is divided into thalassemia major which is transfusion dependent, thalassemia intermedia (of intermediate severity), and thalassemia minor that is asymptomatic. Defects in HBB are the cause of sickle cell anemia (SKCA); also known as sickle cell disease. Sickle cell anemia is characterized by abnormally shaped red cells resulting in chronic anemia and periodic episodes of pain, serious infections and damage to vital organs. Normal red blood cells are round and flexible and flow easily through blood vessels, but in sickle cell anemia, the abnormal hemoglobin (called Hb S) causes red blood cells to become stiff. They are C-shaped and resembles a sickle. These stiffer red blood cells can led to microvascular occlusion thus cutting off the blood supply to nearby tissues. Defects in HBB are the cause of beta-thalassemia dominant inclusion body type (B-THALIB). An autosomal dominant form of beta thalassemia characterized by moderate anemia, lifelong jaundice, cholelithiasis and splenomegaly, marked morphologic changes in the red cells, erythroid hyperplasia of the bone marrow with increased numbers of multinucleate red cell precursors, and the presence of large inclusion bodies in the normoblasts, both in the marrow and in the peripheral blood after splenectomy. Belongs to the globin family. Protein type: Carrier. Chromosomal Location of Human Ortholog: 11p15.5. Cellular Component: hemoglobin complex; extracellular region; cytosol. Molecular Function: haptoglobin binding; protein binding; peroxidase activity; hemoglobin binding; iron ion binding; heme binding; oxygen binding; oxygen transporter activity. Biological Process: receptor-mediated endocytosis; positive regulation of nitric oxide biosynthetic process; nitric oxide transport; response to hydrogen peroxide; oxygen transport; protein heterooligomerization; bicarbonate transport; hydrogen peroxide catabolic process; regulation of blood pressure; blood coagulation; regulation of blood vessel size. Disease: Fetal Hemoglobin Quantitative Trait Locus 1; Beta-thalassemia; Sickle Cell Anemia; Heinz Body Anemias; Beta-thalassemia, Dominant Inclusion Body Type; Malaria, Susceptibility To; Alpha-thalassemia

size1 :

0.05 mL

price1 :

285 USD

size2 :

0.2 mL

price2 :

555