Subunit hemoglobin alfa

Subunit hemoglobin alfa
Struktur sedia ada
PDBPencarian ortolog: PDBe RCSB
Senarai kod PDB

1A00, 1A01, 1A0U, 1A0Z, 1A3N, 1A3O, 1A9W, 1ABW, 1ABY, 1AJ9, 1B86, 1BAB, 1BBB, 1BIJ, 1BUW, 1BZ0, 1BZ1, 1BZZ, 1C7B, 1C7C, 1C7D, 1CLS, 1CMY, 1COH, 1DKE, 1DXT, 1DXU, 1DXV, 1FDH, 1FN3, 1G9V, 1GBU, 1GBV, 1GLI, 1GZX, 1HAB, 1HAC, 1HBA, 1HBB, 1HBS, 1HCO, 1HDB, 1HGA, 1HGB, 1HGC, 1HHO, 1IRD, 1J3Y, 1J3Z, 1J40, 1J41, 1J7S, 1J7W, 1J7Y, 1JY7, 1K0Y, 1K1K, 1KD2, 1LFL, 1LFQ, 1LFT, 1LFV, 1LFY, 1LFZ, 1LJW, 1M9P, 1MKO, 1NEJ, 1NIH, 1NQP, 1O1I, 1O1J, 1O1K, 1O1L, 1O1M, 1O1N, 1O1O, 1O1P, 1QI8, 1QSH, 1QSI, 1QXD, 1QXE, 1R1X, 1R1Y, 1RPS, 1RQ3, 1RQ4, 1RQA, 1RVW, 1SDK, 1SDL, 1SHR, 1SI4, 1THB, 1UIW, 1VWT, 1XXT, 1XY0, 1XYE, 1XZ2, 1XZ4, 1XZ5, 1XZ7, 1XZU, 1XZV, 1Y01, 1Y09, 1Y0A, 1Y0C, 1Y0D, 1Y0T, 1Y0W, 1Y22, 1Y2Z, 1Y31, 1Y35, 1Y45, 1Y46, 1Y4B, 1Y4F, 1Y4G, 1Y4P, 1Y4Q, 1Y4R, 1Y4V, 1Y5F, 1Y5J, 1Y5K, 1Y7C, 1Y7D, 1Y7G, 1Y7Z, 1Y83, 1Y85, 1Y8W, 1YDZ, 1YE0, 1YE1, 1YE2, 1YEN, 1YEO, 1YEQ, 1YEU, 1YEV, 1YFF, 1YG5, 1YGD, 1YGF, 1YH9, 1YHE, 1YHR, 1YIE, 1YIH, 1YVQ, 1YVT, 1YZI, 1Z8U, 2D5Z, 2D60, 2DN1, 2DN2, 2DN3, 2DXM, 2H35, 2HBC, 2HBD, 2HBE, 2HBF, 2HBS, 2HCO, 2HHD, 2HHE, 2M6Z, 2W6V, 2W72, 2YRS, 3B75, 3D17, 3D7O, 3DUT, 3HXN, 3IA3, 3IC0, 3IC2, 3KMF, 3NL7, 3NMM, 3ODQ, 3ONZ, 3OO4, 3OO5, 3OVU, 3P5Q, 3QJB, 3QJC, 3QJD, 3QJE, 3R5I, 3S48, 3S65, 3S66, 3SZK, 3WCP, 3WHM, 4FC3, 4HHB, 4IJ2, 4L7Y, 4M4A, 4M4B, 4MQC, 4MQG, 4MQH, 4MQI, 4MQJ, 4MQK, 4N7N, 4N7O, 4N7P, 4N8T, 4NI0, 4NI1, 4ROL, 4ROM, 4WJG, 4X0L, 4XS0, 6HBW, 5E29, 5E6E, 5EE4, 5HU6, 5JDO, 5KDQ, 5E83,%%s1Y01, 1Z8U, 1BZ1

Pengecam
AliasHBA1, HBA-T3, HBH, hemoglobin subunit alpha 1, METHBA, ECYT7
Pengecam-pengecam luaranOMIM: 141800 MGI: 96015 HomoloGene: 469 GeneCards: HBA1
Kedudukan gen (Manusia)
Kromosom 16
Kr.Kromosom 16[1]
Kromosom 16
Kedudukan gen Subunit hemoglobin alfa
Kedudukan gen Subunit hemoglobin alfa
Jalur16p13.3Mula176,680 bp[1]
Tamat177,522 bp[1]
Corak ekspresi RNA
Bgee
HumanMouse (ortholog)
Top expressed in
  • monocyte

  • darah

  • Sumsum tulang

  • bone marrow cells

  • Plasenta

  • limpa

  • ganglionic eminence

  • muscle of thigh

  • olfactory zone of nasal mucosa

  • apex of heart
Top expressed in
  • Sumsum tulang

  • limpa

  • paru-paru

  • Kantung yolka

  • neural tube

  • Otak tengah

  • jantung

  • korteks serebelum

  • genital tubercle

  • kawasan kulit
More reference expression data
BioGPS
tiada data
Ontologi gen
Fungsi molekul
  • iron ion binding
  • oxygen binding
  • peroxidase activity
  • oxygen carrier activity
  • ikatan ion logam
  • Pengikatan protein plasma
  • heme binding
  • haptoglobin binding
  • organic acid binding
Komponen sel
  • cytosol
  • endocytic vesicle lumen
  • blood microparticle
  • membran
  • extracellular region
  • cytosolic small ribosomal subunit
  • hemoglobin complex
  • extracellular exosome
  • haptoglobin-hemoglobin complex
  • Ekstrasel
Proses biologi
  • positive regulation of cell death
  • protein heterooligomerization
  • oxygen transport
  • receptor-mediated endocytosis
  • bicarbonate transport
  • hydrogen peroxide catabolic process
  • response to hydrogen peroxide
  • cellular oxidant detoxification
  • transport
Sumber:Amigo / QuickGO
Ortolog
SpesiesManusiaMencit
Entrez

3039

15122

Ensembl

ENSG00000206172

ENSMUSG00000069919

UniProt

P69905

Q91VB8

RefSeq (mRNA)

NM_000558

NM_008218

RefSeq (protein)

NP_000508
NP_000508.1
NP_000549.1

NP_001077424

Kedudukan (UCSC)Chr 16: 0.18 – 0.18 Mbtiada data
Carian PubMed[2][3]
Wikidata
Papar/Sunting data manusiaPapar/Sunting data mencit

Subunit hemoglobin alfa[4] ialah sebuah protein hemoglobin yang dikodkan oleh gen HBA1 dalam manusia.[5]

Gen

Kelompok gen globin alfa manusia yang terletak pada kromosom 16 menjangkau kira-kira 30 kb dan merangkumi tujuh lokus: 5'- zeta - pseudozeta - mu - pseudoalfa-1 - alfa-2 - alfa-1 - teta - 3'. Urutan pengekodan alpha-2 (HBA2) dan alpha-1 (HBA1; gen ini) adalah sama. Gen ini berbeza sedikit di wilayah 5' tidak ditranslasikan dan intron, tetapi ia berbeza ketara di wilayah 3' tidak ditranslasikan.[5]

Protein

Dua rantai alfa dengan dua rantai beta membentuk HbA yang merangkumi kira-kira 97% jumlah hemoglobin dalam kehidupan dewasa normal; rantai alfa bergabung dengan rantai delta untuk membentuk HbA-2 yang membentuk baki 3% hemoglobin dewasa bersama dengan hemoglobin janin (HbF).

Kepentingan klinikal

Talasemia alfa terhasil daripada pemadaman setiap gen alfa serta pemadaman kedua-dua HBA2 dan HBA1; beberapa talasemia alfa bukan pemadaman juga telah dilaporkan.[5]

Interaktsi

Subunit hemoglobin alfa telah diperhatikan berinteraksi dengan subunit hemoglobin beta (HBB).[6][7]

Rujukan

  • Subunit hemoglobin beta

Rujukan

  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000206172 - Ensembl, May 2017
  2. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  3. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "HBA1 gene: MedlinePlus Genetics".
  5. ^ a b c "Entrez Gene: HBA1 hemoglobin, alpha 1".
  6. ^ Stelzl U, Worm U, Lalowski M, Haenig C, Brembeck FH, Goehler H, Stroedicke M, Zenkner M, Schoenherr A, Koeppen S, Timm J, Mintzlaff S, Abraham C, Bock N, Kietzmann S, Goedde A, Toksöz E, Droege A, Krobitsch S, Korn B, Birchmeier W, Lehrach H, Wanker EE (September 2005). "A human protein-protein interaction network: a resource for annotating the proteome". Cell. 122 (6): 957–68. doi:10.1016/j.cell.2005.08.029. hdl:11858/00-001M-0000-0010-8592-0. PMID 16169070. S2CID 8235923.
  7. ^ Shaanan B (November 1983). "Structure of human oxyhaemoglobin at 2.1 A resolution". J. Mol. Biol. 171 (1): 31–59. doi:10.1016/S0022-2836(83)80313-1. PMID 6644819.

Pautan luar

  • Entri talasemia alfa di GeneReviews/NCBI/NIH/UW
  • Entri talasemia alfa di OMIM
  • Gambaran maklumat struktur tersedia di PDB bagi UniProt: P69905 (Hemoglobin subunit alpha) di PDBe-KB.
  • l
  • b
  • s
Protein pembawa hem (hemoprotein)
Globin
Hemoglobin
Subunit
Lokus alfa 16:
  • α
    • HBA1
    • HBA2
    • pseudo
  • ζ
    • HBZ
  • θ
    • HBQ1
  • μ
    • HBM
Lokus beta 11:
  • β
  • δ
    • HBD
  • γ
    • HBG1
    • HBG2
  • ε
    • HBE1
Tetramer
tahap
perkembangan:
Embrio
  • HbE Gower 1 (ζ2ε2)
  • HbE Gower 2 (α2ε2)
  • HbE Portland I (ζ2γ2)
  • HbE Portland II (ζ2β2)
Janin
Dewasa
  • HbA (α2β2)
  • HbA22δ2)
  • HbF/Janin2γ2)
berpatologi:
  • HbH4)
  • Barts (γ4)
  • HbS2βS2)
  • HbC (α2βC2)
  • HbE (α2βE2)
  • HbO (α2βO2)
Sebatian
  • Karboksihemoglobin
  • Karbaminohemoglobin
  • Oksihemoglobin/Deoksihemoglobin
  • Sulfhemoglobin
Lain-lain
(manusia)
  • Hemoglobin berglikosil
  • Metemoglobin
Bukan manusia
  • Klorokruorin
  • Eritrokruorin
Lain-lain
manusia:
  • Mioglobin
    • Metmioglobin
  • Neuroglobin
  • Sitoglobin
tumbuhan:
  • Leghemoglobin
Lain-lain
  • Sitokrom
    • Sitokrom b
    • Sitokrom P450
  • Metemalbumin
lihat juga gangguan protein globin dan globulin