PDHA1
PDHA1，全稱(chēng)為丙酮酸脫氫酶復(fù)合體E1亞基α1，常被簡(jiǎn)稱(chēng)為PDHE1-α1。它是細(xì)胞能量代謝中的核心分子之一，能推動(dòng)糖代謝產(chǎn)生的丙酮酸轉(zhuǎn)化為乙酰輔酶A，這是糖酵解與三羧酸循環(huán)之間的關(guān)鍵銜接步驟，直接關(guān)系到細(xì)胞能否高效生成能量。
PDHA1功能異常，易引發(fā)丙酮酸脫氫酶缺乏癥。患者多出現(xiàn)乳酸水平異常升高、神經(jīng)發(fā)育遲緩、運(yùn)動(dòng)能力障礙等表現(xiàn)，部分嚴(yán)重病例還可能伴隨腦損傷、器官功能紊亂等問(wèn)題。目前針對(duì)這類(lèi)疾病的干預(yù)手段包括補(bǔ)充硫辛酸等輔酶類(lèi)物質(zhì)以改善酶活性，也在探索基因治療或小分子藥物修復(fù)蛋白功能。
熱銷(xiāo)產(chǎn)品
驗(yàn)證數(shù)據(jù)
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
PDHA1 Recombinant Monoclonal Antibody (CSB-RA440123A0HU)
驗(yàn)證數(shù)據(jù)
Flow cytometric analysis of PDHA1 expression in HepG2 cells using PDHA1 antibody. Green, isotype control; red, PDHA1.
Immunocytochemical staining of HepG2 cells with PDHA1 antibody. Nuclei were stained blue with DAPI; PDHA1 was stained magenta with Alexa Fluor? 647. Images were taken using Leica stellaris 5. Protein abundance based on laser Intensity and smart gain: Medium. Scale bar: 20 μm.
Western blotting analysis using PDHA1 antibody. Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with PDHA1 antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively.
PDHA1 Antibody (CSB-PA017715LA01HU)
驗(yàn)證數(shù)據(jù)
Immunohistochemistry of paraffin-embedded human colon cancer using CSB-PA017715LA01HU at dilution of 1:100
Immunofluorescent analysis of PC-3 cells using CSB-PA017715LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
PDHA1 Antibodies
PDHA1 for Homo sapiens (Human)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱(chēng) | 種屬反應(yīng)性 | 應(yīng)用類(lèi)型 |
|---|---|---|---|
| CSB-PA017715GA01HU | PDHA1 Antibody | Human,Mouse,Rat | ELISA,WB,IHC,IF |
| CSB-PA247984 | PDHA1 Antibody | Human | ELISA,WB,IHC |
| CSB-PA003727 | PDHA1 Antibody | Human,Mouse,Rat | WB, IHC, ELISA |
| CSB-PA017715LA01HU | PDHA1 Antibody | Human | ELISA, IHC, IF |
| CSB-PA017715LD01HU | PDHA1 Antibody, Biotin conjugated | Human | ELISA |
| CSB-PA017715LC01HU | PDHA1 Antibody, FITC conjugated | Human | |
| CSB-PA017715LB01HU | PDHA1 Antibody, HRP conjugated | Human | ELISA |
| CSB-RA189996A0HU | PDHA1 Recombinant Monoclonal Antibody | Human, Rat | ELISA, WB, IHC, FC |
| CSB-RA440123A0HU | PDHA1 Recombinant Monoclonal Antibody | Human, Mouse, Rat | ELISA, WB, FC, ICC |
PDHA1 Proteins
PDHA1 Proteins for Rattus norvegicus (Rat)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱(chēng) | 來(lái)源 |
|---|---|---|
| CSB-YP017715RA CSB-EP017715RA CSB-BP017715RA CSB-MP017715RA CSB-EP017715RA-B |
Recombinant Rat Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (Pdha1) | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
PDHA1 Proteins for Mus musculus (Mouse)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱(chēng) | 來(lái)源 |
|---|---|---|
| CSB-YP017715MO CSB-BP017715MO CSB-MP017715MO CSB-EP017715MO-B |
Recombinant Mouse Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (Pdha1) | Yeast Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
PDHA1 Proteins for Sus scrofa (Pig)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱(chēng) | 來(lái)源 |
|---|---|---|
| CSB-YP017715PI CSB-EP017715PI CSB-BP017715PI CSB-MP017715PI CSB-EP017715PI-B |
Recombinant Pig Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1) | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
PDHA1 Proteins for Canis familiaris (Dog) (Canis lupus familiaris)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱(chēng) | 來(lái)源 |
|---|---|---|
| CSB-YP017715DO CSB-EP017715DO CSB-BP017715DO CSB-MP017715DO CSB-EP017715DO-B |
Recombinant Dog Pyruvate dehydrogenase E1 component subunit alpha, somatic form (PDHA1) | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
PDHA1 Proteins for Homo sapiens (Human)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱(chēng) | 來(lái)源 |
|---|---|---|
| CSB-YP017715HU CSB-BP017715HU CSB-MP017715HU CSB-EP017715HU-B |
Recombinant Human Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1), partial | Yeast Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
| CSB-EP017715HUb1 | Recombinant Human Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1), partial | E.coli |
| CSB-EP017715HUc0 | Recombinant Human Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1), partial | E.coli |
| CSB-EP017715HU | Recombinant Human Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1), partial | E.coli |
| CSB-EP017715HUc7 | Recombinant Human Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1), partial | E.coli |
| CSB-EP017715HUa2 | Recombinant Human Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1), partial | E.coli |
PDHA1 Proteins for Bos taurus (Bovine)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱(chēng) | 來(lái)源 |
|---|---|---|
| CSB-YP017715BO CSB-EP017715BO CSB-BP017715BO CSB-MP017715BO CSB-EP017715BO-B |
Recombinant Bovine Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1) | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
PDHA1 Proteins for Pan troglodytes (Chimpanzee)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱(chēng) | 來(lái)源 |
|---|---|---|
| CSB-YP397897EQV CSB-EP397897EQV CSB-BP397897EQV CSB-MP397897EQV CSB-EP397897EQV-B |
Recombinant Pan troglodytes Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1) | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
PDHA1 Proteins for Pongo abelii (Sumatran orangutan)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱(chēng) | 來(lái)源 |
|---|---|---|
| CSB-YP728879PYX CSB-EP728879PYX CSB-BP728879PYX CSB-MP728879PYX CSB-EP728879PYX-B |
Recombinant Pongo abelii Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1) | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
PDHA1 Proteins for Macaca fascicularis (Crab-eating macaque) (Cynomolgus monkey)
| 產(chǎn)品貨號(hào) | 產(chǎn)品名稱(chēng) | 來(lái)源 |
|---|---|---|
| CSB-YP808486MOV CSB-EP808486MOV CSB-BP808486MOV CSB-MP808486MOV CSB-EP808486MOV-B |
Recombinant Macaca fascicularis Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (PDHA1) | Yeast E.coli Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
