MMADHC Antibody

中文名稱：

MMADHC兔多克隆抗體
貨號：

CSB-PA875665LA01HU
規(guī)格：

￥440
圖片：
IHC image of CSB-PA875665LA01HU diluted at 1:300 and staining in paraffin-embedded human glioma performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

IHC image of CSB-PA875665LA01HU diluted at 1:300 and staining in paraffin-embedded human prostate cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.

Immunofluorescence staining of HepG2 cells with CSB-PA875665LA01HU at 1:100, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點詳情

產(chǎn)品詳情

產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) MMADHC Polyclonal antibody
Uniprot No.：

Q9H3L0
基因名：

MMADHC
別名：

C2orf25 antibody; cblD antibody; Chromosome 2 open reading frame 25 antibody; CL25022 antibody; Methylmalonic aciduria (cobalamin deficiency) cblD type; with homocystinuria antibody; Methylmalonic aciduria and homocystinuria type D protein antibody; methylmalonic aciduria and homocystinuria type D protein; mitochondrial antibody; mitochondrial antibody; MMAD_HUMAN antibody; Mmadhc antibody; Protein C2orf25; mitochondrial antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Methylmalonic aciduria and homocystinuria type D protein, mitochondrial protein (26-142AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產(chǎn)品，MMADHC Antibody (CSB-PA875665LA01HU)，的標記方式是Non-conjugated。對于MMADHC Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產(chǎn)品名稱	應用
HRP	CSB-PA875665LB01HU	MMADHC Antibody, HRP conjugated	ELISA
FITC	CSB-PA875665LC01HU	MMADHC Antibody, FITC conjugated
Biotin	CSB-PA875665LD01HU	MMADHC Antibody, Biotin conjugated	ELISA

克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產(chǎn)品提供形式：

Liquid
應用范圍：

ELISA, IHC, IF

推薦稀釋比：

Application	Recommended Dilution
IHC	1:200-1:500
IF	1:50-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
Immunofluorescence (IF) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

功能：

Involved in cobalamin metabolism and trafficking. Plays a role in regulating the biosynthesis and the proportion of two coenzymes, methylcob(III)alamin (MeCbl) and 5'-deoxyadenosylcobalamin (AdoCbl). Promotes oxidation of cob(II)alamin bound to MMACHC. The processing of cobalamin in the cytosol occurs in a multiprotein complex composed of at least MMACHC, MMADHC, MTRR (methionine synthase reductase) and MTR (methionine synthase) which may contribute to shuttle safely and efficiently cobalamin towards MTR in order to produce methionine.
基因功能參考文獻：
1. analysis of crystal structure of the globular C-terminal domain of human CblD, which is sufficient for its interaction with MMADHC or CblC, and for supporting the cytoplasmic cobalamin trafficking pathway PMID: 26364851
2. specific regions of MMADHC are involved in differential regulation of adenosylcobalamin and methylcobalamin synthesis PMID: 24722857
3. MMADHC mutations are associated with methylmalonic aciduria and homocystinuria. PMID: 22156578
4. mutations in a gene designated MMADHC (currently named C2orf25) are responsible for the cblD defect in vitamin B12 metabolism; various mutations are associated with each of the three biochemical phenotypes of the disorder PMID: 18385497
相關(guān)疾?。?/div>
Methylmalonic aciduria and homocystinuria type cblD (MMAHCD)
亞細胞定位：

Cytoplasm. Mitochondrion.
組織特異性：

Widely expressed at high levels.
數(shù)據(jù)庫鏈接：

HGNC: 25221

OMIM: 277410

KEGG: hsa:27249

STRING: 9606.ENSP00000301920

UniGene: Hs.5324

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